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DERMATOLOGY > Gen Derm > Flashcards

Gen Derm Flashcards

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1
Q

Heerfordt’s syndrome

A

Variant of sarcoid: Uveoparotid syndrome

  • *facial nerve palsy
  • BIL anterior uveitis
  • Parotid gland enlargement
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2
Q

Lofgren’s syndrome

A

Acute sarcoid

  • fever
  • migratory polyarthritis
  • E nodosum
  • BIL hilar adenopathy
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3
Q

Mikulicz syndrome

A

Sicca symptoms in sarcoid!
complex constellation of symptoms seen in sarcoid and other:
-lacrimal and parotid enlargement=sicca symptomes

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4
Q

Parinaud oculoglandular syndrome

A

Variant of sarcoid: ipsilateral conjunctivitis and parotid gland enlargement

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5
Q

Darier-Roussy disease

A

Sarcoid panniculitis

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6
Q

Lupus pernio

A

Variant of sarcoid as papulonodules on the face

-associated with chronic lung disease and bone cysts

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7
Q

sarcoid panniculitis

A

Darier-Roussy disease

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8
Q

The Laminin formerly known as Laminin-332

A

Laminin 5 (Intermediate filaments)

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9
Q

What is MADDPORES?

A

The pneumonic for EPS! (Elastosis Perforans Serpiginosa)

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10
Q

Name all of the diseases that EPS is found in?

A 
MADDPORES!
Marfans
Acrogeria
Downs
D-penacillamine
Psuedoxanthoma elasticum
Osteogenesis Imperfecta
Rothmund Thompson
Ehlers Danlos 
Scleroderma
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11
Q

What medicine causes EPS?

A

D-penacillamine

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12
Q

A person with Wilson disease got a weird serpiginous thing on their neck, what is this?

A

EPS - this is because D-penacillamine is an increased risk factor for developing EPS

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13
Q

Which genoderm carries an increased risk of developing osteosarcoma?

A

Rothmund Thompson (1/3 of patients get it!) and nothing else does this

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14
Q

p53 is located on which chromasome?

A

the long arm of chromasome 17

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15
Q

the majority of p53 mutations involve which part of the gene?

A

the sequence specific DNA binding domain

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16
Q

Underphosphorylated Rb binds to which transcription factor to prevent the cell from entering S phase?

A

E2F

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17
Q

a connective tissue nevus in Tuberous Sclerosis

a connective tissue nevus is Buschke-Ollendorff syndrome

A

Shagreen Patch

dermatofibrosis leticularis disseminata

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18
Q

Name two common allergans in topical steroid vehicles?

A
  1. propolyne glycol

2. sorbitan sesquidate

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19
Q

Name two common allergans in topical steroid vehicles?

A
  1. propolyne glycol

2. sorbitan sesquidate

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20
Q

How many CMEs do you need a year?

A

50

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21
Q

What lung finding in Birt Hogg Dube

A

spontaneous pneumothorax

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22
Q

carpal ankylosis is pathoneumonic for which syndrome?

A

Adult onset Stills disease (carpal ankylosis is the fusing of the two bones)

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23
Q

generalized pustular psoriasis in pregnancy is also referred to as:

A

Impetigo herpetiformis

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24
Q

Acrokeratosis Verruciformis

A

Darier’s Disease (ATP2A)

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25
Q

B1

A

Thiamine

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26
Q

B2

A

Riboflavin

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27
Q

B3

A

Niacin

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28
Q

B5

A

patothenic acid

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29
Q

B6

A

Pyridoxine

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30
Q

B7

A

Biotin

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31
Q

B9

A

Folic Acid

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32
Q

B12

A

Cobalamin

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33
Q

Thiamine

A

B1

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34
Q

Riboflavin

A

B2

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35
Q

Niacin

A

B3

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36
Q

Pantothenic Acid

A

B5

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37
Q

Pyridoxine

A

B6

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38
Q

Folic Acid

A

B9

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39
Q

Cobalamin

A

B12

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40
Q

Oro-ocular genital syndrome

A

deficiencies in B2 (riboflavin) and B6 (pyridoxine)

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41
Q

Pellagra

A

Vitamin B3 deficiency (Niacin)

Diarrhea 
Dermatitis 
Dementia 
"casal's" necklace
pigmentation of the dorsal hands and feet
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42
Q

Beri Beri

A

Thiamine (B1) deficiency

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43
Q

Sideroblastic anemia

A

Vitamin B6 (Pyridoxine)Deficiency

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44
Q

The genetic form of zinc deficiency

A

Acrodermatitis Enteropathica

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45
Q

The genetic form of biotin deficiency

A

Holocarboxylase Deficiency

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46
Q

Megaloblastic anemia

A

Folic acid (Vitamin B9) deficiency

OR

Cobalamin (Vitamin B12) deficiency

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47
Q

“macrocytosis with hypersegmented neutrophils”

A

pathomnemonic for Folic acid (vitamin b9) deficiency

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48
Q

Which drugs cause PML?

A

Cyclosporine
Mycophenolate mofetil
natalizumab
rituximab

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49
Q

why should people with first degree relatives who have MS not take TNF-alpha inhibitors?

A

TNF inhibitors have increased risk for demyelinating neuropathies

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50
Q

dapsone and thalidomide - which type of neuropathies are associated

A

dapsone - motor neuropathies (reversible)

thalidomide - sensory neuropathies (irreversible)

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51
Q

Dry Beri Beri

A

peripheral neuropathy

found in thiamin deficiency

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52
Q

Wet beri beri

A

congestive heart failure

found in thiamine deficiency

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53
Q

what vitamin deficiency can cause a peripheral neuropathy?

A

thiamine
B1

*dry beri beri

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54
Q

Glossitis

peripheral neuropathy

A

Dry beri beri - thiamine deficiency

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55
Q

polish rice diet

A

thiamine deficiency

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56
Q

coenzyme in carbohydrate metabolism

A

thiamine (b1)

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57
Q

vitamin in collagen formation

A

vitamin c

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58
Q

vitamin that plays a role in the formation of glucose

A

thiamine

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59
Q

oral ocular genital syndrome

A

Vitamin B2 Riboflavin

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60
Q 
magenta-colored tongue
angular chelitis
seb derm
genital dermatitis
photophobia
A

Oral ocular genital syndrome (Vitamin B2 riboflavin deficiency)

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61
Q

deficiency found in celiac sprue

A

vitamin B2 riboflavin

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62
Q

vitamin responsible for production of gluathione

A

Vitamin B2 riboflavin

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63
Q

Vitamin responsible for pellagra

A

Vitamin B3 Niacin

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64
Q 
photosensitive eruption on the face neck and upper chest
diarrhea 
angular chelitis
perianal dermatitis
disorientation
A

Vitamin B3 Niacin

This is Pellagra!

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65
Q

Casals necklace

A

pellagra - vitamin B3 Niacin

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66
Q

vitamin causing a peripheral neuropathy

A

thiamine *vit B1

pyridoxine *vit B6

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67
Q

Vitamin that is a tryptophan precursor amino acid

A

Niacin *vit b3

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68
Q

deficiency associated with a diet composed of entirely corn

A

Niacin *vit B3

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69
Q

deficiency caused by a use of isoniazid (INH)

A

Niacin *vit B3

AND

Pyridoxine *vit B6

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70
Q

vitamin deficiency caused by carcinoid syndrome

A

niacin *vitamin b3

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71
Q

Vitamin def associated with cirrosis and uremia

A

Vitamin B6 Pyridoxine

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72
Q

UVB + what = Vitamin D?

A

7-dehydrocholesterol

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73
Q

foamy white spots on the conjunctiva

A

Bitots spots (vitamin A excess)

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74
Q

What skin findings to pts with Vitamin D deficiency have?

A

alopecia (NO skin rashes!)

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75
Q

lab abnormality in decreased Vitamin K

A

prolonged PT

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76
Q

active form of vitamin D

A

calcitriol

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77
Q

what kind of fatty acids can the body not synthesize?

A

unsaturated FA

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78
Q

spoon shaped nails due to iron deficiency

A

koilonychia

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79
Q

Patients on Targretin should not use which common household product?

A

DEET! For some reason Bexarotine (targretin) combines with DEET to increase DEET toxicity

*Alitretinoin also causes this side effect

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80
Q

A patient treated got a topical “beauty cream” from a doctor to treat his psoriasis, and after working in the yard in the summer he gets sweaty diaphoretic, ect. What happened?

A

DEET toxicity. The psoriasis med was likely targretin (bexarotine, binds RXR receptors), and it interacts with DEET to cause DEET toxicity

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81
Q

Which topical retinoid is applied 3-4 times per day to treat CTCL?

A

Targretin (bexarotene)

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82
Q

Which topical retinoid is used to treat Kaposi’s sarcoma?

A

Ali-tretinoin (9-cis trans retinoic acid) it is one of the naturally derived

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83
Q

another name for alitretinoin

A

9-cis-retinoic acid

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84
Q

Which two topical retinoids are the only ones which bind to the RXR receptor?

A

Bexarotene (Targretin) -RXR exclusively

Alitretinoing (9-cis-retinoic acid) RXR and RAR

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85
Q

Which two topical retinoids are category X?

A

targretin (bexarotene) and tazorac (tazarotene)

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86
Q

Which key cellular component of the development of acne is blocked by topical retinoids?

A

TLR-2 (which interacts with p acnes to form the microcomedome, the precursor lesion to inflammatory papules and comedomes)

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87
Q

Granular cell tumor is derived from what tissue?

A

Neural tissue

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88
Q

Melanocytes appear in which week of gestation?

A

10 weeks

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89
Q

Which two cutaneous B cell lymphomas have a good prognosis?

A

Follicle center, and marginal zone

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90
Q

Which B cell lymphoma is most closely related to Borriela infection?

A

Marginal zone lymphoma

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91
Q

BCL-2
FOX-1
MUM
are all markers for which skin disease?

A

Primary cutaneous diffuse large B cell lymphoma, leg type

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92
Q

Marker that is positive in almost all of the Follicle center B cell lymphomas

A

BCL-6

also CD10

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93
Q

what is the wavelength used in NB-UVB therapy?

A

311nm

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94
Q

which cutaneous B cell lymphoma is associated with Borriella?

A

Marginal

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95
Q

Most common cosmetic preservative to cause ACD?

A

Quaternium-15

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96
Q

contact allergen found in vaccines

A

thimerosal

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97
Q

What do you patch test for if trying to find an allergy to hydrocortisone?

A

Tixocortol-21-pivalate

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98
Q

What do you patch test for if you want to find an allergy to triamcinolone?

A

Budesonide

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99
Q

What do you patch test for if you want to find an allergy to fluocinonide?

A

Budesonide

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100
Q

What do you patch test for if you want to find an allergy to clobetasol or betamethasone?

A

Hydrocortisone-17-butyrate

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101
Q

Dont eat which two foods if you have a bad allergy to poison ivy?

A

Cashews or mangos

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102
Q

What is Baboon syndrome?

A

An ACD on the buttocks caused by ingestion of some sort of pesto pasta containing cashews in a person who is allergic to poison ivy

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103
Q

What family is poison ivy a part of?

A

Anacardiacea

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104
Q

Wavelenth of narrowband UVB

A

311-313

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105
Q

PUVA therapy peaks at what wavelength:

A

352

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106
Q

wavelength 290-320 is what?

A

UVB spectrum

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107
Q

Excimer laser is what wavelength?

A

308

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108
Q

UVA2 spectrum is what wavelengths?

A

320-340

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109
Q

UVA1 wavelengths

A

340-400

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110
Q

what wavelengths do you do PDT at?

A

400-700 (blue and red light)

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111
Q

contraindication for phototherapy

A

thiazides, doxycycline, and lupus

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112
Q

most common DNA photoproduct:

A

cyclobutane-pyrimide dimers (CPD)

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113
Q

most frequent cyclobutane-pyrimide dimer:

A

T-T

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114
Q

signature DNA mutation related to UVB

A

C–>T (cyctoside changed to thymine)

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115
Q

what is the mechanism of action of mutations from:
UVA
UVB

A

UVA- from ROS

UVB -from formation of dimers

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116
Q

pts with pemphigoid gestationis have an increased risk of what post partum?

A

graves disease

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117
Q

why do first generation antihistimines make you drowsy?

A

because they are LIPOPHILIC - and thus can cross the blood brain barrier

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118
Q

How can first gen antihistamines cross the blood brain barrier?

A

they are LIPOPHILIC

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119
Q

Fexofinadine trade name, and prodrug name

A

Allegra, its prodrug is 1st gen terfinadine

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120
Q

which second gen antihistamine is not proven safe to use in pregnancy?

A

fexofenadine (Allegra)

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121
Q

which second gen H1 blocker is metabolized by CYP3A4 inhibitor and why does that matter?

A

Loratidine (Claritin) - because this is one of the major inhibitors in the liver where it is metabolized and theoretically could interact with another drug like a macrolide or grapefruit juice to increase blood levels of loratidine = cardiac arythmias

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122
Q

which second gen H1 blocker is the metabolite of a first gen H1 blocker (and which one is it?)

A

zyrtec (certirizine) is the metabolite of hydroxizine (Atarax)

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123
Q

which antihistamine do you take in pregnancy?

A

chlorpheniramine!

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124
Q

Gene mutated in both atopic derm and ichtyosis vulgaris

A

Fillagrin

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125
Q

what are dennie morgan lines?

A

the two lines below the canthus in atopic derm patients

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126
Q

Contact urticaria is what kind of hypersensitivity?

A

Type I

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127
Q

what is the most common underlying malignancy seen in someone with erythema gyratum repens?

A

lung cancer

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128
Q

Bacterial phage that causes bullous impetigo

A

Phage II, type 71

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129
Q

bacteria causing impetigo

causing bullous impetigo

A

impetigo = staph >strep

bullous impetigo= ONLY staph

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130
Q

what are pastias lines?

A

seen in scarlett fever: they are dark lines of confluent purpura found in the axillary and antecubial folds

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131
Q

sharply bordered, punched out ulcer with yellow leathery psudomembrane

A

cutaneous diptheria

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132
Q

difference between erysipelas and erysipeloid

A

erysipelas = strep infection

erysipeloid= erysipelothrix, fish handlers disease (also a gram positive bacteria tho)

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133
Q

Madura foot with red grains is caused by what bacteria?

A

Actinomadura

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134
Q

Actinomycetoma (Madura foot) with white grains caused by what bacteria?

A

Nocardia

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135
Q

Madura foot caused by yellow grains

A

Streptomyces

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136
Q

PAPA syndrome

A

pyogenic arthritis
PG
acne

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137
Q

SAPHO syndrome

A 
Sweet's with:
synovitis
acne
pustules
hyperostosis
osteitis
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138
Q

synonym for Intravascular Papillary Endothelial Hyperplasia

A

Massons tumor

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139
Q

Prodrome of fever and backache

  • macules/papules initially on face, spreads to trunk and extremities
  • papules then turn to vessicles with central umbilication
  • *lesions are all the same age
A

-Variola (smallpox)

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140
Q

Variola virus causes:

A

Smallpox

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141
Q

Name the findings in POEMS syndrome

A 
Polyneuropathy
Organomegaly
Endocrine abnormalities
Monoclonal gammopathy
Skin findings (glomeroloid hemangioma)
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142
Q

What vascular neoplasm do you see in POEMS syndrome?

A

Glomeruloid Hemangioma

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143
Q

In what two syndromes do you find a Glomeruloid Hemangioma?

A

POEMS and Castlemans syndrome

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144
Q

Angiokeratoma corporis diffusum is found most famously in what disease?

A

XLR Fabry disease

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145
Q

Multiple clustered angiokeratomas in a bathing suit distribution

A

Angiokeratomas Corporis Diffusum

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146
Q

Multiple angiokeratomas on the fingers and toes, associated with chillblains and acrocyanosis

A

Angiokeratosis of Mibelli

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147
Q

A group of little papules that look like cherry angiomas, usually present from birth, on the arms and legs

A

Angioma circumsciptum

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148
Q

Is TH1 or TH2 seen in chronic atopic derm?

A

TH1 for chronic atopic derm

TH2 for acute atopic derm

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149
Q

Is TH1 or TH2 seen in chronic atopic derm?

A

TH1 for chronic atopic derm

TH2 for acute atopic derm

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150
Q

Mutations in FILLAGRIN (epidermal cornification protein) are seen in what two disorders?

A

Atopic Dermatitis
&
Icthiosis Vulgaris

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151
Q

Hertoge sign

A

Absense or thinning of the lateral eyebrows in atopic derm

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152
Q

Which antihistimine has T cell suppressor therapy?

A

Cimetidine

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153
Q

keratins found in suprabasal layer

A

K1/K10

AND

highly expressed K9 in suprabasal layer of palmoplantar epidermis

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154
Q

what site is K9 highly expressed in?

A

Suprabasal layer of palmoplantar epidermis

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155
Q

BASAL layer keratins

A

K5/14

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156
Q

palms and soles keratinocytes

A

K6/17

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157
Q

Name two molecules in the cadherin family

A

Desmoglein

Desmocollin

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158
Q

Mutation in steatocystoma multiplex

A

Keratin 17

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159
Q

Steatocystoma multiplex can be seen in what genoderm?

A

Pachonychia congenita Type II

mutation in Keratin 6a, 17

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160
Q

What is the mutation in Pachonychia congenita Type II?

A

Keratin 6a, 17

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161
Q

What skin finding is associated with Pachyonychia Congenita due to mutation in Keratin 17?

A

Steatocystoma multiplex

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162
Q

What embryonic layer is hair derived from?

A

Ectoderm

With the exception of the dermal papilla which is derived from mesoderm!!!

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163
Q

What layer is the dermal papilla derived from?

A

Mesoderm

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164
Q

What is the widest diameter of the hair follicle called?

A

Critical line of auber

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165
Q

Which part of the hair segment does the bulk of mitotic activity occur?

A

The inferior segment right below the critical line of auber

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166
Q

Which part of the hair do the melanocytes that determine hair color live?

A

The inferior segment (in the bulb)

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167
Q

Name the layers of the hair from the outer-most to the inner most

A 
Glassy membrane
Outer root sheath
Henle (IRS)
Huxley (IRS)
Cuticle (IRS)
Hair shaft cuticle
Cortex
Medulla
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168
Q

Where in the hair do you find the outer root sheath?

A

The entire hair follicle!

It kertatinizes in the infundibular portion and has tricholemmal characterization in the isthmus and the inferior segment

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169
Q

Where is the inner root sheath found in the hair follicle?

A

Found ONLY in the lower segment (not found in the isthmus or the infundibulum)

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170
Q

Which layer of the hair follicle contains the majority of the hair KERATINS

A

The corseted

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171
Q

Which layer of the hair follicle maintains the integrity of the hair fibers?

A

The cuticle layer

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172
Q

What is the lower portion of the hair follicle?

A

Extends from the bulge of the isthmus (insertion point of arrest or pili) down to the bulb of the follicle

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173
Q

What portion of the hair follicle contains stem cells?

A

The bulge

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174
Q

Where is the insertion point for the arrector pili muscle?

A

THE BULGE

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175
Q

What term in the hair follicle is used to describe a collection of mesenchymal cells which protrudes into the hair bulb?

A

The dermal papilla

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176
Q

Which hair cycle is the growth phase?

A

Anagen

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177
Q

How many hairs are in anagen at one time?

A

85%

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178
Q

Which hair loss syndrome has miniaturization of telogen and catagen follicles?

A

Alopecia areata

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179
Q

On dermascopy, what would you see in AA?

A

“Exclamation point hairs”

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180
Q

Alopecia with varying lengths of broken hair

A

Trichotillomania

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181
Q

Deformed hair shafts

A

Trichomalacia

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182
Q

Which hair disorder do you find trichomalacia?

A

Trichotillomania

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183
Q

In which type of alopecia would you find an increased number of telogen hairs, normal number of follicles, increased vellous hairs

A

Androgenetic alopecia

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184
Q

Comma hairs, or corkscrew hairs

A

Tinea capitis

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185
Q

Bamboo hair

A

Trichorrhexis invaginata - Netherton syndrome

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186
Q

Tufted hair

A

Multiple hair shafts emergin from 1 follicle - see in scarring alopecia like folliculitis decalvans and dissecting cellulitis

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187
Q

Name all of the herpes viruses

A 
HHV1- oral herpes
HHV2-genital herpes
HHV3-VZV(chicken pox, shingles)
HHV4-EBV, mono, Gianotti Crostii, Burkitts, Hodgkins
HHV5- CMV
HHV6-Roseola
HHV7-Pityriasis Roseola 
HHV8- Kaposi's, Castlemans
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188
Q

Which HHV causes PR?

A

HHV7

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189
Q

Which HHV causes Kaposi

A

HHV8

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190
Q

Which HHV causes Burkitts

A

HHV4

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191
Q

Which HHV causes roseola?

A

HHV6

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192
Q

Which HHV causes VZV

A

HHV3

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193
Q

Which HHV causes Gionotti Crosti?

A

HHV4

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194
Q

Which HHV causes CMV?

A

HHV 5

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195
Q

Causes of EM

A

HSV
Medication induced: NSAIDS, anticonvulsants
MYCOPLASMA

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196
Q

Difference between EM major and minor

A

No oral lesions in EM minor

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197
Q

Which HLA class is most strongly associated with SCLE?

A

HLA-B8

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198
Q

Botulinum toxin binds to heavy chain or light chain?

A

Heavy chain

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199
Q

The antigen in DH

A

Transglutaminase

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200
Q

What thing do you want to check in a baby with neonatal lupus?

A

ECG - 50% of these babies will have permanent 3rd degree heart block!

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201
Q

What are the chances the next baby of a sibling with neonatal lupus will also get neonatal lupus?

A

25%

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202
Q

Which type of porokeratosis has the highest risk of turning into a squam?

A

Linear porokeratosis

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203
Q

Trichoeps
Cylindromas
Spiradenomas
BCCs

A

Brooke Speigler

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204
Q

Brooke Speigler

A

Trichoeps
Cylindromas
Spiradenomas
BCCs

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205
Q

Brooke-Speigler gene defect

A

CLYD (cylindromatosis)

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206
Q

Classic triad: alopecia, diaper dermatitis, diarrhea

A

Acrodermatitis enteropathica (zinc deficiency)

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207
Q

Gene for acral melanoma

A

C-KIT

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208
Q

Mucosal melanoma

A

C-kit

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209
Q

Uveal/Ocular melanoma

A

GNAQ

Highly aggressive metastatic ocular is BAP1

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210
Q

Non sun damaged skin/intermittent high intensity sun exposed melanoma

A

BRAF

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211
Q

Lentigo maligna gene

A

C-KIT

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212
Q

Gene defect in chronic sun exposed skin melanoma

A

C-KIT

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213
Q

Metastatic ocular melanoma gene

A

BAP-1

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214
Q

Familial melanoma gene

A

CDKN2A, BAP

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215
Q

Gene mutation in XP melanoma

A

PTEN

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216
Q

Gene mutation in congenital nevi

A

NRAS

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217
Q

Spitz nevi/spitzoid melanoma gene mutation

A

HRAS

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218
Q

Melanin needs what element to make pigment

A

Copper

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219
Q

What specialty should you refer someone to if they begin making multiple halo nevi after age 40 suddenly?

A

Optho! May indicate ocular melanoma (GNAQ)

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220
Q

What HLA type has the highest risk of developing psoriasis?

A

Cw6

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221
Q

How much of your hair is in anagen phase at once?

A

95%

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222
Q

How much of your hair is in telogen phase at once?

A

5%

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223
Q

Name three drugs associated with gingival hyperplasia

A

Phenytoin
Nifedipine
Cyclosporine

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224
Q

What type of monoclonal gammopathy is associated with scleromyxedema

A

Lambda light chain

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225
Q

An accessory tragus represents an anomaly of what anatomic structure?

A

First brachial arch

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226
Q

Butchers warts are caused by which HPV type?

A

HPV 2 and HPV 7

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227
Q

How many Diabetes patients have NLD?

A

3%

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228
Q

How many NLD patients have diabetes?

A

30%

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229
Q

HPV type Butcher’s warts

A

2, 7

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230
Q

HPV flat warts

A

3, 10

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231
Q

HPV type plain warts

A

3,10

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232
Q

HPV type Hecks disease

A

13, 32

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233
Q

HPV type condyloma acuminata

A

6, 11

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234
Q

HPV type verrucous carcinoma

A

6,11

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235
Q

HPV type EDV

A

Too many to count! 2,3,5,-8-10, 12, 14

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236
Q

HPV type common wart

A

1,2,4

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237
Q

HPV type plantar wart

A

1

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238
Q

HPV type Bowenoid papulosis

A

16,18

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239
Q

What two kinds of proteins does the HPV virus encode?

A

E (early) and L (later)

E6 and E7
L1 and L2

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240
Q

What does the HPV E6 and E7 protein encode?

A

Viral DNA replication, which decreases p53 function (E6) and bind Rb (E7)

Vs L (late proteins), which encode viral STRUCTURAL PROTEINS

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241
Q

Which HPV proteins does the Gardisil vaccine encode?

A

L (late proteins) 6, 8, 11, 18

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242
Q

PCT has what finding in the urine?
How about stool?
RBCs?

A

UD (uroporphyrinogen) - urine
Isocopro - stool
RBC - normal

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243
Q

PCT enzyme defect

A

Uroporphyrinogen Decarboxylase

Urine dazzles pink

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244
Q

Triggers for aquired PCT

A 
Hemochromatosis
Estrogen
HIV
HCV
Alcohol
Polychlorinated hydrocarcbons
Iron overload
C282Y gene
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245
Q

What gene mutation is associated with aquired PCT?

A

C282Y

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246
Q

What type of monoclonal gammopathy do you see in scleromyxedema?

A

IgG - lambda light chain

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247
Q

What is the enzyme defect in EPP? (Erythropoietin Protoporphyria)?

A

Ferrochelatase

E(PP) –>F (errochelatase)

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248
Q 
In EPP, what enzymes are found in the:
Urine 
Stool
RBC:
Plasma
A

Urine: normal
Stool: proto
RBC: proto
Plasma: flouresces

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249
Q

Which is the only porphyria to have normal urine findings?

A

EPP (Ferrochelastase). *Ferra Faucet is a Pro

Urine: normal
Stool: proto
RBC: proto

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250
Q

Which porphyria presents with photosensitivity with burning
Healing with waxy scars
Porphyrin gallstones
Hepatic damage

A

EPP

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251
Q

Inheritance pattern of PCT

A

AD

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252
Q

Inheritance pattern of EPP

A

AD

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253
Q

What is another name for Gunthers disease?

A

CEP (Congenital Erythropoetic Porphyria)

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254
Q

Congenital Erythropoetic Porphyria is also called:

A

Gunthers disease

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255
Q

Inheritance pattern for Gunthers disease

A

AR (Congenital Erythropoetic Porphyria) CEP

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256
Q

Enzyme defect in Gunthers disease?

A

Uroporphyrinogen III cosynthase

This is CEP, Congenital Erythropoetic Porphyria

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257
Q

Which porphyria presents with erythrodontia

A

CEP (congenital erythropoetic porphyria), defect in Uroporphyrinogen III cosynthase (Ur Teeth r Colored*)

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258
Q 
In CEP, name the enzymes found in the:
Urine
Stool
RBCs
Plasma
A 
(CEP enzyme defect is Uro III Cosynthase)
Urine: uro 
Stool:copro
RBC: uro
Plasma: flouresce
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259
Q 
Which porphyria presents with:
Extreme photosensitivity 
Bulla heals with intense mutilated scarring
Hypertrichosis
Erythrodontia
Hemolysis (anemia)
Red urine, stains diapers
Increased risk of skin CA
A

CEP (Congenital erythropoetic porphyria)

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260
Q

Porphyria associated with erythrodontia

A

CEP

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261
Q

Baby has red stains in their diapers

A

CEP (Congenital Erythropoetic Porphyria)

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262
Q

Baby has red teeth and red diapers

A

CEP

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263
Q

Baby has red teeth and red stains in diapers, what enzyme must be deficient?

A

This must CEP (congenital erythropoetic porphyria), defect in the enzyme Uroporphyrinogen III Cosynthase

+++uro found in the urine and blood

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264
Q

What type of cancers are kids with CEP at risk for?

A

Skin cancers

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265
Q

Which porphyria carries the most dramatic increased risk of skin cancers?

A

CEP

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266
Q

Which porphyria usually requires a bone marrow transplant?

A

CEP

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267
Q

What wavelengths are absorbed intensely by porphyrins?

A

400-410 (the Soret band)

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268
Q

A child with bulla, sunburns, bad mutilated scars has a splenectomy, which porphyria does this sound like?

A

CEP (findings would also include red teeth, red diapers, hemolysis)

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269
Q

Name the only two AR inherited porphyrias?

A

CEP
&
HEP

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270
Q

Which porphyria has the same enzyme defect as PCT and what is it?

A

HEP (Hepatoerythropoietic Porphyria), and the defect is in Uroporphyrinogen Decarboxylase

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271
Q

Which porphyria is essentially an overlap between PCT and CEP?

A

HEP

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272
Q

In which porphyria is phlebotomy not effective?

A

HEP

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273
Q

Enzyme defect in AIP (acute intermittent porphyria)

A

Pophobilinogen deaminase (abdomen is painful, Please Barbituates Doc)

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274
Q 
In AIP, name the findings in the:
Urine
Stool 
RBC
Plasma
A 
Acute Intermittent Porphyria:
Urine: ALA, PBG
Stool: normal 
RBC: normal
Plasma: normal
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275
Q

Which porphyria has no skin findings?

A

AIP

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276
Q

Which porphyria has neurologic and psychiatric problems along wtih abdominal pain?

A

AIP - no skin findings!!!

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277
Q

What do you find in the urine of someone with psychiatric problems, abdominal pain, and a family history of AD porphyria?

A

Aminolevulinic acid (ALA) and Porphobilinogen (PBG)

This is Acute intermittent Porphyria! (AIP). There are no skin findings, only neuro and psychiatric findings associated with abdominal pain

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278
Q

Name some triggers of AIP

A 
Drugs (barbituates)
Stress
Fasting
Alcohol
Hormonal changes
Infections
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279
Q

How do you treat AIP attacks? (Acute intermittent porphyria)

A

Remove the trigger, glucose load, and hematin infusion

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280
Q

What do you find in the stool of an AIP patient (acute intermittent porphyria)

A

Nothing!

Only find ALA or PBG in the urine, nothing in the stool, RBC, or plasma

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281
Q

Name the findings in PAPA syndrome

A

Pyogenic Arthritis
Pyoderma gangrenosum
Acne

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282
Q

Mutation in PAPA syndrome

A

CD2BP1 (pyrin-interacting protein) like the familial med fever, muckle wells, ect

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283
Q

Patient presents with:

  • Pyogenic Arthritis
  • Pyoderma gangrenosum
  • Acne
A

PAPA syndrome

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284
Q

PG
Acne
Arthritis

A

PAPA syndrome:
Pyogenic Arthritis
Pyogenic granuloma
Acne

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285
Q 
Patient presents with:
Acne
Synovitis
Palmoplantar pustulosis
Hyperostosis
Osteitis
A 
SAPHO syndrome:
Synovitis
Acne (congoblata)
Palmoplantar pustolosis
Hyperostosis
Osteitis
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286
Q

Name the findings in SAPHO syndrome

A 
Synovitis 
Acne
Palmoplantar pustulosis
Hyperostosis
Osteitis
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287
Q

Another name for Chronic recurrent multifocal osteomyelitis

A

SAPHO syndrome

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288
Q

What is the most common location of inflammatory bone changes in SAPHO syndrome?

A

Sternoclavicular joint

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289
Q

Name three systemic syndromes associated with acne:

A

PAPA syndrome
SAPHO syndrome
HAIR-AN syndrome

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290
Q

What are the findings in HAIR-AN syndrome?

A

HypernAdrogenism, Insulin resistance, Acanthosis Nigricans

This is listed in Jain as an acne related condition like PAPA/SAPHO

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291
Q

Gene mutation in Lichen Sclerosis

A

ECM-1 (extracellular matrix protein)

*also found in Lipoid Proteinosis

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292
Q

What derm condition is due to a defect in Extra-cellular Matrix protein?

A

ECM-1 Lichen sclerosis, Lipoid Proteinosis

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293
Q

Enzyme defect in AIP?

A

PBG (porphobilinogen deaminase)

AIP (Abdomen is painful….) BPD (please barbituates D/c) *bc barbituates are a trigger

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294
Q

What is the enzyme defect in Varigate Porphyria?

A

PPO (protoporphyrinogen oxidase)

ViPs get the PPO health insurance?

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295
Q 
In Varigate Porphyria, what are the findings in the:
Urine:
Stool:
RBCs:
Plasma:
A

Urine: ALA/PBG
Stool: proto
RBC: normal
Plasma: flouresce

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296
Q

What wavelenth does the plasma of a person with Varigate Porphyria flouresce at?

A

626nm

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297
Q

Hereditary Coproporphyria has a defect in what enzyme?

A

CPO (coproporphyrinogen oxidase)

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298
Q 
In Hereditary Coproporphyria, what are the findings in the:
Urine:
Stool:
RBCs:
Plasma:
A 
HCP:
Urine: ALA/PBG
Stool: copro
RBC: normal
Plasma: doesnt flouresce?
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299
Q 
In Hepatoerythropoietic Porphyria, what are the findings in the:
Urine:
Stool:
RBCs:
Plasma:
A

HEP-
Urine: uro
Stool: uro
RBCs: proto

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300
Q

Sarcoid presenting in children

A

Blau syndrome

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301
Q

What is Blau syndrome?

A

Child sarcoid

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302
Q

Which of the periodic fever syndromes do you NOT treat with anakinra?

A

Familial Mediterranean Fever - treat this one with colchicine instead!

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303
Q

What do you treat Familial Mediterranean fever syndrome with?

A

Colchicine! (You treat the other ones like Muckle Well and TRAPs with anakinra)

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304
Q

Child with: extreme photosensitivity
Bulla with subsequent mutilated scarring
Hypertrichosis

A

CEP

…also these kids have the erythrodontia and the red urine stains in the diapers

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305
Q

Child with erythrodontia

A

CEP (Congenital Erythropoetic Porphyria)

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306
Q

Name the clinical findings of CEP (Congenital Erythropoetic Porphyria)

A

Extreme sun photosensitivity
Bullae with mutilated scars
Erythrodontia
Red stains of urine in the diaper

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307
Q

What blood finding are kids with CEP at risk for?

A

Hemolysis

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308
Q

Kid with bulla and massive scaring and coproporphyrinogen in their stool. What enzyme is defective?

A

Uroporphyrinogen III cosynthase

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309
Q

Photosensitivity with burning
Heals with waxy scars
Liver enzymes elevated
Has to have gallbladder removed

A

EPP (Erythropoetic Protoporphyria)

-they gell porphyrin gallstones which leads to hepatic damage

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310
Q

Porphyrin gallstones

A

EPP

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311
Q

Tense bulla,erosions, milia and scarring on sun-exposed skin
Hypertrichosis on temples
Iron overload
Facial hyperpigmentation

A

PCT

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312
Q

Erbium:YAG laser can cause damage to which part of the eye?

A

Cornea (because erbium targets water)

Lasers that target pigment will damage the retina

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313
Q

Purely ocular form of cicatricial pemphigoid

A

Beta-4 integrin

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314
Q

Malignancy associated form of cicatricial pemphigoid

A

Laminin 5

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315
Q

What is the first/most common physical exam finding of cicatricial pemphigoid?

A

Oral involvement

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316
Q

Antibodies to collagen 7 causes what disease?

A

EBA

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317
Q

Defective gene for collagen 7 causes which disease?

A

Dystrophic EB

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318
Q

Drug most likely to cause BP?

A

Furosemide

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319
Q

Most common drug to cause linear IgA

A

Vancomycin

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320
Q

What size is the BPAG2 molecule responsible for linear IgA?

A

97kD

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321
Q

How big is BPAG2 causing BP?

A

180kD

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322
Q

Antibody associated with sclerodermoid changes in DM

A

Anti-Ku

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323
Q

Cardiac and poor prognosis in DM seen with what antibody

A

Anti-SRP

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324
Q

What are the most common cancers associated with DM in:
Men
&
Women:

A

Men - lung and GI

Women - ovarian

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325
Q

HLA-B51

A

Bechets

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326
Q

What HLA type is associated with Bechets

A

HLA-B51

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327
Q

Chronic candidal infections, vitiligo, thyroid problems

A

APECED (autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy)

Mutation in the AIRE gene

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328
Q

Family and genus of poison ivy

A

Anacardiacea family

Toxicodendron genus

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329
Q

Family and genus of poison sumac

A

Anacardiacea family

Toxicodendron genus

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330
Q

UVB spectrum in nm

A

290-320

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331
Q

Mast cells are found in greatest density in what part of the skin?

A

Papillary dermis

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332
Q

Acrokeratosis Paraneoplastica

A

Basex sign (multiple keratotic papules on the hands and feet as a sign of SCC Of the upper aerodigestive system, like esophageal SCC)

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333
Q

Multiple acral keratotic papules on the hands and feet in a patient with SCC of the esophagus

A

Acrokeratosis Neoplasticism (Bazex sign)

*Bazex syndrome is an XLD syndrome with lots of BCC, follicular atrophoderma an d

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334
Q

What layer of the epidermis are keratins 5/14 produced in?

A

Basal layer

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335
Q

What keratins are produced in the basal layer?

A

K5/14

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336
Q

What keratins are produced in the spinous layer?

A

Keratin 1/Keratin 10

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337
Q

What two diseases are due to decrease in lamellar granules?

A

Flegels disease
&
Harlequin Ichthyosis

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338
Q

What is the #1 component of the cornified envelope (stratum corneum)

A

Loricrin

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339
Q

What is the target of light-based acne therapy?

A

Coproporphyrin III

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340
Q

What androgen is produced by the adrenal glands?

A

DHEA

  • testosterone is produced by the gonads, and then converted to DHT (most potent androgen) by 5-alpha reductase in the body
  • DHEA-S is a weak androgen produced by the adrenal glands
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341
Q

Where are the androgen receptors located in the pilosebaceous unit?

A

In the basal layer of the sebaceous gland, and also on the outer root sheath

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342
Q

Name a side effect of isotretinoin

A

Diffuse idiopathic skeletal hyperostosis

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343
Q

Diffuse idiopathic skeletal hyperostosis is a rare side effect of what medication?

A

Isotretinoin

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344
Q

Do not take what kind of medications while on accutane

A

Tetracyclines (Can cause pseud

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345
Q

What vitamin excess can cause an acne form eruption?

A

B12

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346
Q

Second most common place to get osteolytic bone lesions in acne fulminans?

A

Ankle

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347
Q

Follicular occlusion triad/tetrad

A

Acne
Dissecting cellulitis of the scalp
Pilonidal cyst
HS

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348
Q

Name a few adrenal androgens

A

1-hydroxyprogesterone

DHEA-S

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349
Q

Lab finding in PCOS

A

Increased FSH/LH ratio

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350
Q

Name the chemical that most commonly causes industrial acne

A

2,3,7,8 tetrachlorobenzodioxin

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351
Q

Most common cause of industrial acne

A

Insolvable cutting oils

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352
Q

Distribution of industrial acne

A

Malar cheeks, retroauricular region, scrotum

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353
Q

Cephalic neonatal pustulosis

A

Neonatal acne

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354
Q

Acne causing a swollen, “wooden” ruddy face

A

Morbihans disease

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355
Q

Mutation in PAPA syndrome

A

Purine interacting protein - CD2 binding protein 1

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356
Q

PAPA syndrome mutation

A

CD2 binding protein 1

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357
Q

CD2 binding protein 1

A

PAPA syndrome(pyrine binding protein like in Muckle Wells, familial cold urticaria)

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358
Q

What infection do you worry about in someone who takes anti-biopics long term (acne-form eruption)

A

Gram-Negative Folliculitis (centrofacial pustules, perinasal)

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359
Q

Most specific Ab in drug induced lupus

A

Anti-histone Ab

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360
Q

Family of plants that causes phytophotodermatitis

A

Apiaceae (celery, parsley, parsnip, hogweed)

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361
Q

What is the most important component of a Baker Gordon peel?

A

Croton oil (a deep phenol peel)

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362
Q

Name an anti microbial peptide that results in the decreased risk of secondary infection in psoriasis

A

Human beta-defensin 2 (HBD2)

Also skin-derived anti-leukoproteinase SKALP, and secretory leukocyte protease inhibitor SLPI

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363
Q

What cell envelope protein is upregulated in psoriasis?

A

Involucrin

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364
Q

Strongest association with early onset psoriasis

A

Cw6

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365
Q

Associated with early onset psoriasis

A

B13, DR7, B17, B57

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366
Q

HLA associated with psoriatic arthritis

A

B27

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367
Q

HLA associated with guttate psoriasis

A

B13, B17, CW6

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368
Q

What is the Wornoff ring

A

Blanching area around a PSO plaque due to PGE-2

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369
Q

Risk factor for pustular psoriasis?

A

Hypocalcemia

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370
Q

Pustular psoriasis associated with what HLA type?

A

HLA-B27

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371
Q

What cytokine is high in the synovium of patients with psoriatic arthritis?

A

TNF-alpha (Why TNF-A inhibitors work)

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372
Q

Von Zumbusch

A

Generalized pustular psoriasis

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373
Q

What’s the most common drug that causes PR?

A

ACE-Inhibitor (Captopril, Lisinopril)

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374
Q

What hastens the resolution of PR?

A

Erythromycin

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375
Q

What is the classic type of PRP?

A

Type I

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376
Q

What’s the most common type of PRP in children?

A

Type VI (Type III is the classic but Type VI is the most common)

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377
Q

Most common type of arthritis in psoriatic arthritis

A

Asymmetric oligoarthritis

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378
Q

Most common cause of flare of PRP

A

Phototherapy

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379
Q

Pathopneumonic for Reiters syndrome

A

Circulate balanitis

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380
Q

Name two findings in Reiters syndrome

A

Keratoderma blenorrhagicum

Circulate Balanits

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381
Q

What should you avoid if you have a latex allergy?

A

Avocados

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382
Q

Most common allergic reaction to what color tattoo pigment

A

Red tattoo pigment

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383
Q

Most common (pigmented) fixed drug reaction to which drug

A

Tetracycline

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384
Q

Most common non pigmented fixed drug cause

A

Pseudoephedrine

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385
Q

Most common fixed drug in children

A

Bactrim

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386
Q

1 cause of pityriasis amniacea

A

Psoriasis

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387
Q

What percent of psoriatic arthritis patients have psoriatic arthritis?

A

30%

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388
Q

Does psoriatic arthritis correlate with psoriasis disease severity?

A

Yes

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389
Q

HLA type associated with psoriatic arthritis

A

HLAB27

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390
Q

Collection of neutrophils in the stratum spinosum in psoriasis

A

Micropustule of Kogoj

Microabcesses of Munro and in the stratum corneum

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391
Q

Micropustules of Kogoj

A

Collections of neutrophils in the stratum spinosum in psoriasis

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392
Q

Topical retinoid used for treatment of psoriasis

A

Tazorac (tazarotene)

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393
Q

What type of light therapy is best used in guttate psoriasis flares

A

Broad-band UVB (vs NVUVB for other types of psoriasis)

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394
Q

What is the only systemic retinoid used for psoriasis

A

Acitretin

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395
Q

In which two types of psoriasis is acitretin effective mono therapy

A

Erythrodermic and pustular psoriasis

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396
Q

Name of the sign in psoriasis where you scrape off a scale to find pinpoint bleeding

A

Auspitz sign

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397
Q

Treatment of choice for impetigo herpetiformis (pustular psoriasis in pregnancy)

A

Early delivery, prednisone

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398
Q

Which type of PRP is the only focal type?

A

Type IV

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399
Q

What does type V PRP entail?

A

Atypical juvenile form - basically PRP with sclerodermoid changes on the hands and feet

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400
Q

Most common drug causing PR

A

ACE-inhibitors

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401
Q

Drug will hasten the resolution of PR

A

Erythromycin

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402
Q

New gene defect found in alopecia areata

A

ULBP3

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403
Q

Lab finding in patients with familial alopecia areata

A

Thrombocytopenia

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404
Q 
#1 symptom of erythroderma 
AND
#1 extracutaenous finding of erythroderma
A
  1. Pruritis

2. Lymphadenopathy

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405
Q

Most common cause of erythroderma in healthy patients
AND
Most common cause of erythroderma in HIV patients

A
  1. Psoriasis

2. Drug

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406
Q

Where does CARP usually start?

A

Inframammary

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407
Q

Treatment for CARP

A

Minocycline

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408
Q

Variant of CARP on the neck with vertically oriented lesions with wrinkling

A

Psuedoatrophoderma colli

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409
Q

Psuedoatrophoderma colli

A

Variant of CARP on the neck with vertical plaques and wrinkled skin

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410
Q

Cytokines and cell types in chronic atopic derm

A

TH1 cells and IFN-y

411
Q

Cytokines and cell types in acute AD

A

TH2 cells, and IL-4, IL-5, IL-12, IL-13

412
Q

Mutation associated with early onset atopic derm

A

Fillagrin

413
Q

Hertoge sign

A

Loss of lateral eyebrows in atopic derm

414
Q

Ocular findings in atopic dermatitis

A

Posterior subcapsular cataracts

Keratoconus (elongation of the cornea)

415
Q

Keratoconus

A

Elongation of the cornea (see in atopic derm)

416
Q

Food allergy is what type of allergic reaction

A

Type I

417
Q

Atopic dermatitis one week before period

A

Progesterone dermatitis (occurs with the LUTEAL phase - 1 weeks before period and resolves a few days after menses)

418
Q

Most common cause of ACD worldwide

A

Nickel

419
Q

Most common cause of ACD in the USA

A

Poison ivy (nickel is worldwide)

420
Q

ACD is what type of hypersensitivity reaction?

A

Type IV (delayed type)

421
Q

What is the most common site of an ICD?

A

The hands

Face is #2!

422
Q

UVA spectrum

A

320-400nm

423
Q

How long after contact with allergic do you get a phytophotodermatitis?

A

24-72 hours

424
Q

What two things are required to create a phytophotodermatitis?

A

UVA (320-400nm) and a fucocoumarins

425
Q

Phytophotodermatitis caused by which spectrum of light?

A

UVA (320-400)

426
Q

What is berloque dermatitis?

A

Phytophotodermatitis from a cologne containing bergamot oil

427
Q

What chemical causes Beloque dermatitis

A

Berganot oil (found in men’s colognes)

428
Q

What flower is the #1 cause of ACD in florists?

A

Tulip!

429
Q

What nail product is the most common cause of eyelid dermatitis?

A

Tosylamide

430
Q

Tosylamide

A

Nail product that is the most common cause of eyelid dermatitis

431
Q

What fabric finisher is one of the most commons causes of clothing dermatitis?

A

Formaldehyde

432
Q

What clothing dye is one of the most common causes of clothing dermatitis?

A

Disperse blue dyes 106 and 124

433
Q

What is EBA most commonly associated with?

A

Crohn’s disease/IBD (most commonly) >multiple myeloma

434
Q

Antigen in EBA

A

Type VII collagen (major component of anchoring fibrils)

435
Q

Aquired diseases against Type VII collagen

A

EBA

Bullous Lupus

436
Q

HLA type present in 97% of DH patients

A

HLA-DQ2

437
Q

Most common autoimmune association with dermatitis herpetiformis (besides celiac)

A

Hashimoto’s thyroiditis> IDDM > pernicious anemia

438
Q

Name three foods gluten is NOT found in

A

Oats, rice, corn

IS found in wheat rye and barley

439
Q

Antigenic byproduct of gluten

A

Gliadin

440
Q

Skin antigen in DH, and gut antigen found in dermatitis herpetiformis

A

Skin: Transglutaminase 3
Gut: Transglutaminase 2

(Side note - Transglutaminase 1 is mutated in Lamellar Ichthyosis, and transglutaminase 1 is found in the granular layer and is the enzyme responsible for the cornified cell envelope)

441
Q

Dermatitis herpetiformis has what kind of infiltrate in the dermal papilae?

A

NEUTROPHILIC

442
Q

How many patients with DH have SYMPTOMATIC IBD?

A

20%

But *90% have some form of celiac on biopsy, its just not always active

443
Q

Dermatitis Herpetiformis is almost indistinguishable in real life from what other blistering disorder?

A

LABD

444
Q

Linear IgA bullous dermatosis in childhood

A

Chronic Bullous Dermatosis of Childhood

445
Q

What does DH look like on path?

A

Granular IgA deposits in the dermal papillae on DIF. On H&E will be a neutrophilic infiltrate in the dermal papillae

446
Q

What serologic test has a high sensitivity

A

Anti-endomysial

You would think it would be anti-gliadin but that one actually give high false positives

447
Q

Treatment of choice for dermatitis herpetiformis

A

Dapsone

Only treats the skin, has no effect on the GI tract

448
Q

What is second line treatment for Dapsone if it is not tolerated in linear IgA and DH?

A

Sulfapyridine (good response with less hemolysis risk)

449
Q

What DH treatment controls both skin and GI disease and is the only way to decrease risk of a MALT lymphoma?

A

Gluten free diet. (Dapsone only treats the skin in DH)

450
Q

DH patients are at risk for what kind of cancer?

A

MALT lymphoma

451
Q

What substance should you avoid in DH to prevent a flare?

A

Iodide

452
Q

SJS/TEN can have antibodies against what cell protein?

A

Desmoplakin I

453
Q

Eosinophilic accesses seen in:

A

Pemphigus Vegetans

454
Q

Anti-dsDNA, anti-Smith

A

SLE, Lupus nephritis

455
Q

Anti-dsDNA

A

SLE, lupus nephritis, *early onset *correlates with disease activity

456
Q

Anti-rRNP

A

Neuropsychiatric SLE

457
Q

Anti-Ro (SSA)

Anti-La (SSB)

A

Mild systemic lupus
SCLE
Neonatal lupus - congenital heart block

458
Q

Anti-histone

A

Drug induced lupus

459
Q

Anti-KU

A

Polymyositis

460
Q

Anti-ssDNA

A

Risk of SLE in DLE

461
Q

What antibody indicates increased risk of SLE in DLE patients

A

SsDLE

462
Q

99% of patients with neonatal lupus with congenital heart block have this antibody

A

Anti-Ro

463
Q

75% of SCLE patients have this antibody

A

Anti-Ro

464
Q

Highly specific antibody for lupus nephritis

A

DsDNA

465
Q

Antibody indicating an increased risk in DLE patients to develop SLE

A

SsDNA

466
Q

Two antibodies highly specific for SLE

A

DsDNA and anti-Smith

467
Q

Antibody highly specific for SCLE

A

Anti-Ro

468
Q

Antibody found in neuropsychiatric lupus

A

rRNP

469
Q

Name most common location for venous stasis leg ulcer

A

Medial supramalleolar region

470
Q

Degos Disease

A

Malignant atrophic papulosis (form of vascular disorder with red papules that evolve into porcelain white scars on the extremities)

471
Q

Malignant atrophic papulosis

A

Degos disease

472
Q

Dowling-Degos

A

Reticulated pigmented anomaly of the flexures

473
Q

What is the initial presenting sign of scleroderma (systemic sclerosis) in >50% of patients?

A

Pitting edema of the digits. The other 50% is Raynauds

474
Q

Systemic Sclerosis (Scleroderma) antibodies:

A

Anti-centromere
Anti-topoisomerase (Scl-70)
Anti-RNA polymerase

475
Q

Which systemic sclerosis/scleroderma antibody is associated with pulmonary HTN?

A

Anti-centromere (this one is also most closely associated with CREST)

476
Q

Which scleroderma antibody is associated with pulmonary fibrosis?

A

Anti-Scl (anti-topoisomerase). *this is the one also most closely associated with systemic sclerosis in general

477
Q

Which scleroderma antibody is most closely associated with severe skin involvement and renal crisis?

A

Anti-RNA polymerase

478
Q

Anti-topoisomerase

A

Also known as anti-Scl: marker for systemic sclerosis/scleroderma

479
Q

Anti-Fibrillarin

A

Associated with internal organ involvement for systemic sclerosis

480
Q

Name the findings in CREST syndrome

A 
Calcinosis cutis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasias
481
Q

Name the antibodies found in Scleroderma

A

Anti-centromere : CREST, pulmonary hypertension
Anti-topoisomerase (anti-RNP): Sscl, pulmonary fibrosis
RNA Polymerase: skin, kidneys
Fibrillan : a/w internal organ damage

482
Q

What is the nail finding in severe progressive systemic sclerosis

A

Pterygium inversum unguis (extension of the hyponychium on the undersurface of the nail plate)

483
Q

Most common site of visceral disease in systemic sclerosis (scleroderma)

A

GI (90%)

  • associated with significant morbidity but almost no mortality
  • the most common cause of death in Sscl is pulmonary
484
Q

What medication will decrease the risk of a renal crisis in systemic sclerosis?

A

ACE-I

485
Q

New biomarker for skin and lung involvement in scleroderma/systemic sclerosis

A

CXCL4

486
Q

What type of morphea is most common in adults? In children?

A

Adults: plaque type morphe
Children: linear morphea

487
Q

What cell marker do you see in both morphea and scleroderma

A

TGF-B *stimulates fibroblast production

488
Q

Morphea is associated with what organism in Europe and Japan?

A

Borriela afzelii, Boriella garinii

489
Q

Which antibodies are a/w linear morphea in children?

A

Anti-ssDNA (*important bc a/w significant morbidity)

490
Q

Brunstein Perry vs Perry Romberg

A

Brunstein Perry- cicatricial pemphigoid limited to head and neck with scarring alopecia & w/o occular findings
Perry-Romberg- unilateral atrophy of the face d/w morphea

491
Q

Ss-DNA antibody

A

A/w linear morphea in children
&
A/w progression of DLE to SLE

492
Q

What anti-body is characteristic of linear morphea in children?

A

ssDNA (sidenote it is also associated with progression of DLE to SLE)

493
Q

Morphea of the face leading to hemiatrophy

A

Perry-Romberg

494
Q

Morphea of the scalp

A

En Coup de Sabre

495
Q

Atrophoderma of Pasini

A

Sharp “cliff drop” of normal skin (this is a type of morphea)

496
Q

Linear atrophoderma of Moulin

A

More benign localized variant of atrophoderma of Pasini, a type of morphea

497
Q

Linear form of atrophoderma (type of morphea)

A

Linear atrophoderma of Moulin

498
Q

What antibodies do all morphea patients lack that scleroderma patients have?

A

Anti-Scl70 (topo-isomerase I)
Anti-centromere

*morphea has anti-topoisomerase II! NOT 1

499
Q

Treatment of choice for moderate to severe morphea

A

MTX

500
Q

High U1RNP titers are seen in what auto-immune disease

A

Mixed Connective tissue disease MCTD

501
Q

Antibody marker in MCTD

A

HIGH titers of U1RNP. Also see a speckled pattern ANA

502
Q

U3RNP
U1RNP
What are they?

A

U3RNP - same this as anti-fibrillan. Seen in Systemic sclerosis a/w internal organ involvement

U1RNP - high titers seen in MCTD

503
Q

Type I keratins -
which ones are they?
are the acidic or basic?
What chromosome are they derived from?

A

Type I keratins are acidic. K9-28. Chromosome 17

504
Q

Type II keratins-
Which are they
Are they acidic or basic
What chromosome are they derived from?

A

K1-8
Basic
Chromasome 12

505
Q

K1/K10 found in what layer of the skin

A

Stratum spinulosum (and really anything suprabasilar)

506
Q

K1,K9 found where?

A

Palmoplantar skin

507
Q

Which keratins are increased in warts in immunosuppressed persons?

A

K4,13

508
Q

Name ALL the lab findings in Chung-Strauss

A

Increased IgE
Increased MPO (myeloperoxidase antibody -this is actually p-anca)
P-Anca

509
Q

What does ANCA stand for?

And what are they antibodies against?

A

Anti-neutrophil cytoplasmic antibodies

They are auto-antibodies against various lysosomal enzymes

510
Q

Another name for P-ANCA

A

Anti-myeloperoxidase antibody

511
Q

Another name for C-ANCA

A

Anti-proteinase 3 (PR-3)

512
Q

Anti-proteinase 3

A

C-ANCA (Wegeners)

513
Q

Anti-Myeloperoxidase

A

P-ANCA (Churg Strauss)

514
Q

Small vessel vasculitis with skin nodules, livedo
Granulomatous vasculitis of the lungs (asthma,allergic rhinitis)
Can affect GI tract, heart
- Does this have P-ACNA or C-ANCA?

A

P-ANCA this is Churg Strauss

515
Q

Systemic vasculitis involving respiratory tract
Granulomatous vasculitis involving kidneys
Mucosal involvement - oral ulcers, friable gingival
Skin ulcers and nodules looks like PG

Does this have a P-ANCA or a C-ANCA lab value?

A

C-ANCA this is Wegeners

516
Q

Which vasculitis can have red friable gingival, oral ulcers, and ulcers on the body which resemble PG?
Can also affect kidneys

A

Wegeners (P-ANCA/Anti-proteinase-3)

517
Q

Which types of cryoglobulinemia are associated with RF?

A

Types II,III (the mixed cryoglobulinemias)

518
Q

What underlying associations are found in cryoglobulinemia types II,III

A

HCV, autoimmune disorders

519
Q

What clinical findings do you see with types II, III cryoglobulinemia?

A

Palpable purpura, glomerulonephritis, peripheral neuropathy

520
Q

Which cryoglobunemias are mixed?

A

Types II, III

521
Q

What is the difference between II, III type cryoglobulinemia?

A

Type II - Monoclonal IgM, Polyclonal IgG

Type III- Polyclonal IgM, Polyclonal IgG

522
Q

What treatment worsens the peripheral neuropathy found in cryoglobulinemia Type II/III?

A

Interferon

523
Q

What treatment should you not give someone who is having peripheral neuropathy associated with a cryoglobulinemia?

A

Interferon (of note, peripheral neuropathy occurs with types II, III)

524
Q

Which type of cryoglobulinemia do you get a peripheral neuropathy?

A

Types II, III

525
Q

Which type of cryoglobulinemia is associated with HCV?

How about autoimmune diseases like lupus?

A

All types II/III

526
Q

What type of antibodies are found in cryoglobulinemia type I?

A

Monoclonal IgM>IgG

*and remember NO rheumatoid activity

527
Q

In which type of cryoglobulinemia is rheumatoid factor not associated?

A

Type I

528
Q

What underlying association is found in cryoglobulinemia type I?

A

Lymphoproliferative disorders

529
Q

Which type of cryoglobulinemia are leukemias associated with?

A

Type I

530
Q

Which type of cryoglobulinemia is Raynauds, purpura, acrocyanosis, and arterial thrombosis found in?

A

Type I

531
Q

Name some clinical findings of Type I cryoglobulinemia?

A

Raynauds, arterial thrombosis, purpura, acrocyanosis

532
Q

What do you treat atrophe Blanche in anti-phospholipid syndrome with?

A

Anti-malarial

533
Q

What is the most specific antibody found in anti-phospholipid syndrome. How about the most common?

A

Specific- anti-beta2-glycoproteins

Most common - anti-cardiolipin

534
Q

How long after starting heparin do you see heparin induced thrombocytopenia?

A

5-10 days

535
Q

What kind of mutation is associated with erythromelalgia in myeloproliferative disorders?

A

JAK-2

536
Q

Gene defect in paroxysmal nocturnal hemiglobinuria

A

PIG-A gene, treat with eculizumab

537
Q

Cancer a/w acanthosis nigricans

A

GI adenocarcinoma (gastric)

538
Q

Cancer a/w aquired angioedema

A

Lymphoproliferative disease

539
Q

Aquired diamond shaped scale on the legs (aquired ichythiosis) a/w with what cancer

A

Hodgkins and NHL (same as aquired angioedema)

540
Q

Cicatricial localized loss of hair a/w with what kind of cancer

A

Metastatic Breast (called alopecia neoplastica)

541
Q

Primary amyloidosis looks like what and is associated with what malignancy

A

*perioribital pinch purpura

A/w multiple myeloma

542
Q

Bazex sign

A

Also called acrokeratosis neoplastica
*a/w upper aerodigestive tract Squamish

Can occur on the ears also

543
Q

What chemical do you test in the urine if you suspect someone has a carcinoid tumor

A

5-HIAA (hydroxyindoleactetic acid), a serotonin metabolite

544
Q

Flushing
Pellagra-like dermatitis
Erythema

A

Carcinoid syndrome

545
Q

Erythema gyratum repens a/w what malignancy

A

Bronchogenic carcinoma

546
Q

Sudden growth of downy hair in an adult a/w what cancer

A

Hypertrichosis lanuginosa acquisita

*a/w lung and colon (b/c a hairy mans chest covers both)

547
Q

Cancer a/w necrolytic migratory erythema

A

Pancreatic (alpha-cell tumor)

548
Q

What type of pancreatic cancer is a/w necrolytic migratory erythema

A

Alpha-cell tumor

549
Q

NXG a/w what type of cancer?

A

Myeloma, occasionally a paraproteinemia

550
Q

Sign of Leser-Trelat a/w what cancer

A
  • suddern eruption of SKs

* a/w gastric, colon, breast

551
Q

Sweets syndrome a/w what malignancy

A

AML

552
Q

Tripe palms

A
  • acanthosis palmaris
  • a/w Lung cancer (if only palms)
  • but if it also has acanthosis nigricans then it is still gastric cancer
553
Q

Trousseau sign

A

*migratory thrombophlebitis, a/w pancreatic cancer

554
Q

Sister Mary Joseph Nodule

A

Nodule in the belly button a/w stomach, bowel cancer

555
Q

Eruptive xanthomas a/w with what condition?

A

Diabetes

556
Q

What type of scleroderma is related to DM?

A

Scleroderma of Buschke (Type III scleredema)

557
Q

What is madarosis

A

Alopecia of the lateral 1/3 of eyebrows (seen in Hypothyroidism)

558
Q

What is necrolytic acral erythema seen in?

A

Hep C

559
Q

Hep C patient has hyperpigmented hyperkeratotic lesions on the hands and feet, what is this?

A

Necrolytic acral erythema

560
Q

Nephrogenic systemic fibrosis a/w what condition?

A

ESRD

561
Q

NSF is seen in ESRD patients with exposure to what chemical?

A

Gadolinium based contrast material

562
Q

Who do you not want to give gadolinium based contrast material to? And why?

A

ESRD patients, bc it will cause NSF

563
Q

Glossitis and pinch purpura see in what

A

Amyloidosis

564
Q

A patient on chronic hemodialysis gets carpal tunnel and back pain - what should you suspect

A

Hemodialysis associated amyloidosis *deposition of beta-2-microglobulin into synovial membranes

565
Q

Protein in hemodialysis associated amyloidosis

A

Beta-2-microglobulin

566
Q

Beta-2-microglobulin

A

Type of amyloid found in hemodialysis associated amyloidosis

567
Q

Amyloid causing cardiomyopathy, inherited type and aquired

A

ATTR/TTR (transthyretin) which is a protein that transports thyroxine and retinol

568
Q

Protein see in familial amyloidosis

A

TTR

569
Q

Melkerson-Rosenthal syndrome

A

Oreo-facial granulomatosis

Triad of facial nerve palsy, fissured tongue, granulomatous chelitis (swelling of gums and lips)

570
Q

Eosinophilic Granuloma

A

Localized benign form of Langerhans Cell Histtocytosis

571
Q

Feature of a poor prognosis in LCH?

A

BRAF V600E

572
Q

BRAF V600E

A

Indicates a poor prognosis in LCH

573
Q

What common progenitor cell do all the histiocytosis disorders share?

A

CD34+

574
Q

First line treatment for Staph Scalded Skin Syndrome

A

Clindamycin

575
Q

Basal ganglia calfications

A

Cockayne Syndrome

576
Q

Most common cause of drug induced dermatomyositis

A

Hydroxyurea (can also be caused by statins)

577
Q

Mechanics hands

A

Strongly associated with anti-syntheses syndrome (form of dermatomyositis)

578
Q

Palmar papules in dermatomyositis associated with what antibody?

A

Anti-CADM-140 *cadbury eggs in the palms

579
Q

What is the PRP Dermatomyositis overlap syndrome called?

A

Wong-type dermatomyositis

580
Q

Juvenile Dermatomyositis associated with severe vasculitis is called

A 
Bankers variant juvenile dermatomyositis
-cutaneous ulceration
Muscle infarction
GI perforation
Widespread calcinosis 
Severe course with poor prognosis

. *hemorrhaging money

581
Q

Child with juvenille DM gets rapid cutaneous ulceration, muscle infarction, perforating bowel, widespread calcinosis, what is this called?

A

Banker’s variate- juvenille DM

582
Q

What finding in DM is actually a good prognostic factor?

A

Raynauds

583
Q 
Raynauds
Mechanics Hands
Interstitial lung disease
Non-erosive arthritis
Anti-synthetase antibodies
Dermatomyositis
A

Anti-synthetase syndrome! *boards favorite

584
Q

Name the findings in anti-synthetase syndrome

A 
DM
Raynauds
ILD
Mechanics hands
Non-erosive arthritis
Anti-synthetase antibodies
585
Q

What is a clinical difference between drug induced hydroxyurea caused Dermatomyositis and non-hydroxyurea induced?

A

Hydroxyurea induced DM NEVER has muscle involvement(and it can start really late - like 60 months after initiation) and non-hydroxyurea ALWAYS has muscle involvement (starts 2 months after the drug)

586
Q

Most common cause of death in dermatomyositis?

A

Malignancy

587
Q

In DM, which usually occurs first - skin or muscle findings?

A

Skin findings usually precede muscle findings by 2-3 months

588
Q

Cicatricial pemphigoid with scalp involvement, scarring alopecia, and no mucosal involvement

A

Brunsting-Perry Variant

589
Q

Brunsting-Perry

A

Variant of cicatricial pemphigoid with no mucosal involvement, but head and neck involvement with scarring alopecia

590
Q

First line treatment for eosinophilic pustular folliculitis (Ofujis)

A

Indomethacin

591
Q

Leading infectious cause of deafness

A

CMV infection of the newborn

592
Q

Blueberry muffin baby
Leading cause of deafness
Owl eye nucleous

A

CMV

593
Q

What can be the presentation of CMV in HIV patients?

A

Lower extremity ulceration
GI erosions
Perianal erosion
Chorioretinitis

594
Q

Complication of Roseola

A

Febrile seizures

595
Q

Castlemans is caused by what virus?

A

HHV-8

596
Q

What virus is primary effusion lymphoma associated with

A

HHV8 (same as Castlemans, Kaposis)

597
Q

What structure in the body (skin) is the target of HPV?

A

Basal layer

598
Q

What cryo temperature do you need to get to destroy:
BCC/SCC
Wart
Melanocytes

A

BCC/SCC: -50
Wart: -20
Melanocytes: -5

599
Q

People with EDV have malignant transformation of warts to SCC in what percentage of patients?

A

50%

600
Q

HPV types Hecks disease

A

13, 32

601
Q

HPV types 13, 32 are what disease?

A

Hecks Disease

602
Q

In EDV, what is the most common HPV types that will progress into SCC?

A

5,8

603
Q

Langerhans cell stains and markers

A

S100+, CD1A+, Langerin (CD207)

*CD207 is the most specific, stains Birbeck granules

604
Q

50% of NXG patients have what kind of complication

A

Opthalmic

605
Q

Send all NXG patients to what specialty

A

Optho (>50% of them get ocular complications)
&
HemeOnc (>80% have an IgG monoclonal gammopathy)

606
Q

> 80% of NXG patients have what finding?

A

IgG monoclonal gammopathy

607
Q

What type of monoclonal gammopathy do 80% of NXG patients get?

A

IgG

608
Q

Location of multicentric reticulohistiocytosis

A

Head, dorsal hands, periungal “coral bead” appearance (papules along periungal region)

609
Q

What other organ systems are affected in multicentric reticulohistiocystosis

A

Mutilating arthritis
Nasopharyngeal involvement
Solid organ malignancy in >30%

610
Q

Solid organ malignancy found in what percentage of multicentric reticulohistiocytosis?

A

30%

611
Q

Most common skin site for Rosai-Dorfman disease?

A

Eyelid>malar cheek

612
Q

S100 is negative in all non-langerhans cell histiocytosis except which two diseases?

A

Rosai-Dorfman & Indeterminate cell histiocytosis (ICH)

613
Q

What clinical findings make up the xanthoma disseminatum triad?

A

Cutaneous xanthomas
Mucosal xanthomas
Diabetes insipidus

614
Q

What lab findings are found in xanthoma dissiminatum

A

Normolipemic
Monoclonal gammopathy
Plasma cell Dysplasia

615
Q

Monoclonal gammopathy associated with plane xanthoma

A

IgG

616
Q

Monoclonal gammopathy associated with Sweets

A

IgA

617
Q

Monoclonal gammopathy associated with Primary amyloidosis (AL)

A

IgG

618
Q

Monoclonal gammopathy associated with NXG

A

IgG kappa

619
Q

Monoclonal gammopathy a/w Scleredema

A

IgG kappa

620
Q

Monoclonal gammopathy a/w Scleromyxedema

A

IgG Lambda

621
Q

Monoclonal gammopathy a/w Pyoderma gangrenosum

A

IgA

622
Q

Monoclonal gammopathy a/w EED

A

IgA

623
Q

Monoclonal gammopathy a/w POEMS syndrome

A

IgA, IgG

624
Q

Monoclonal gammopathy a/w Waldenstroms macroglobulinemia

A

IgM

625
Q

Which familial hyperlipidemias have eruptive xanthomas?

A

Type I, IV, V. (Types 2-3 have tuberous and tendinous xanthomas)

626
Q

Which familial hyperlipidemias do NOT carry an increased risk for coronary artery disease?

A

Type I, IV, V (the ones who have increased triglycerides, eruptive xanthomas)

*its like the triglycerides seep out into the skin and into the pancreas so they dont have time to mess with the heart

627
Q

Which types of familial hyperlipidemias have a creamy top layer in the serum?

A

All of the ones with increase triglycerides - so Type I, IV, V

628
Q

Name the defect in familial hyperlipidemia type I

A

Decrease in lipoproteins lipase (LPL) or Apolipoprotein CII defect

629
Q

Which familial hyperlipidemias have increased risk for pancreatitis

A

Type I, V (same ones that are associated with eruptive xanthomas)

630
Q

Eruptive xanthomas,
DM
Obesity
Alcoholism

A

Type IV hyperlipidemia

631
Q

Xanthoma striatum palmare associated with what type of hyperlipidemia

A

Type III (Apoprotein E)

632
Q

Defect in Type IV hyperlipidemia

A

Increased production of VLDL

633
Q

Defect in types II, III familial hyperlipidemia

A

LDL receptor

634
Q

In which familial hyperlipidemias do you see xanthelasma?

A

Types II, III

635
Q

Hundreds of xanthomas with FLEXURAL predilection, NORMOLIPEMIC, associated with diabetes insipidus

A

Xanthoma disseminatum (can be associated with monoclonal gammopathy too)

636
Q

Besides hyperlipidemia, what other condition can you see xanthomas associated with?

A

Primary biliary cirrhosis

637
Q

Triglycerides must be greater than what number for eruptive xanthomas to form?

A

> 3000

638
Q

Tendinous xanthomas are usually found on what structure and are found in which familial hyperlipidemia?

A

Found on Achilles’ tendon

Usually associated with Type II hyperlipidemia

639
Q

Dysbetalipoprotienemia is also known as which familial hyperlipidemia as the pathopnuemonic characteristic is what finding?

A

Type III familial hyperlipidemia,

Xanthoma striatum palmare

640
Q

What are the preformed ingredients released from mast cells in urticaria?

A

CHT* having a chat

Chymase
Tryptase
Histamine
Heparine

641
Q

Newly formed mediators released from mast cells during urticarial reaction

A

Protaglandin D2
Leukotriene C4/D4/E4
Platelet activating factor

642
Q

Name the causes of immunologic urticaria

A

IgE mediated (Type I hypersensitivity reaction)
Complement-dependent
Immune complex deposition (serum sickness)
Autoantibodies (anti-IgE, anti-FceRI) *chronic urticaria

643
Q

What drugs are most often implicated in urticaria

A 
Antibiotics
NSAIDs
Allopurinol 
Sulfa
Opiates
Radiocontrast
Polymyxin B
644
Q

ACE-I (like captopril) causes angioedema through unregulated generation of what substance?

A

Bradykinin

645
Q

What does CI-INH do?

A

Prevents spontaneous activation of the complement system (serine protease inhibitor)

646
Q

Which complement factor is low in all types of hereditary and aquired angioedemas?

A

C4

*C3 is always normal!

647
Q

What are the natures of the levels of C1-INH in type 1 vs type 2 hereditary angioedema?

A

Type 1= deficient C1-INH

Type 2= normal to high levels of C1-INH, they are just dysfunctional

648
Q

Is C1q elevated, normal, or low in hereditary angioedema?

A

NORMAL C1q in hereditary angioedema (*it is low in aquired angioedema)

649
Q 
What would your labs show in hereditary angioedema?
C4
C3
C1q
C1-INH
A

Decreased C4
Normal C3
Normal C1q
Decreased C1-INH

650
Q

What androgens do you treat hereditary angioedema patients with?

A

Danazol Stanazol

651
Q

Treatments for hereditary angioedema

A

Anti-androgens including danazol and stanazol
Fresh frozen plasma before surgery
C1-INH

652
Q

What lab value is decreased in Aquired angioedema that is normal in hereditary angioedema

A

Decreased C1q

653
Q

C1q is decreased in which: acquired angioedema vs hereditary angioedema?

A

C1q is decreased in aquired angioedema

654
Q

Which is the only type of angioedema in which the C1-INH level is normal or even increased?

A

Hereditary angioedema Type II (where the C1-INH is dysfunctional, instead of type 1 where it is absent)

655
Q

Which aquired angioedema is associated with lymphoproliferative disorders?

A

Aquired angioedema Type I

656
Q

Type I aquired angioedema is associated with what other diseases?

A

Lymphoproliferative disorders/ B cell lymphoma, multiple myeloma ect

657
Q

Which type of angioedema requires more C1-INH during an attack: aquired or hereditary angioedema?

A

Aquired angioedema requires quite a bit more C1-INH during an attack

658
Q

Type of tumor associated with necrolytic migratory erythema

A

Pancreatic tumor (alpha-cell tumor), and if the patient is losing weight and gets DM it is probably a glucagonoma

659
Q

New onset severe intertrigo could be linked to what kind of tumor?

A

Pancreatic tumor (necrolytic migratory erythema)

660
Q

What percentage of NXG patients have a monoclonal gammopathy, and what is it?

A

80%

IgG

661
Q

Which conditions have an IgG gammopathy and what type are they?

A

NXG - IgG kappa

Scleromyxedema - IgG lambda

662
Q

Anti-Ku is what kind of protein?

A

DNA repair protein (p70/p80 nucleoli protein)

663
Q

DM antibody a/w calicinosis cutis

A

P140

664
Q

Relapsing polychondritis a/w antibodies against which type of collagen?

A

Collagen type II

665
Q

1 cause of mortality in patients with relapsing polychondritis

A

Pneumonia (due to involvement of the respiratory cartilage

666
Q

Syndrome a/w relapsing polychondritis

A

MAGIC syndrome (mouth and genital ulcers with inflamed cartilage)= Bechets +relapsing polychondritis

667
Q

What is MAGIC syndrome?

A

Mouth and genital ulcers with inflamed cartilage

I.e. = Bechets + relapsing polychondritis

668
Q

Anti-matrilin-1 (extracellular matrix protein)

A

Relapsing Polychondritis

669
Q

MAGIC syndrome has antibodies against what type of collagen?

A

Type II Collagen

*bc it is basically Bechets plus relapsing polychondritis (and polychondritis is against type II collagen

670
Q

Systemic sclerosis has increased expression of what type of proteins?

A

Extracellular matrix proteins (ECM) from dermal fibroblasts

671
Q

Systemic Sclerosis has increased deposition of which type of collagen?

A

Type III collagen

672
Q

What percent of lichen sclerosis patients will develop into SCC?

A

5%

673
Q

What other autoimmune diseases is lichen sclerosis associated with?

A

Pernicious anemia

Thyroid disease

674
Q

Tumor in argentaffin cells

A

Mid gut tumor, gives you carcinoid syndrome.

675
Q

What do you treat the head and neck flushing of carcinoid syndrome with?

A

Cyproheptadine

676
Q

What do you look for in the urine of people with carcinoid syndrome?

A

Increased 5-HIAA levels (hydroxyindoleacetic acid)

677
Q

What drugs worsen AIP?

A

Barbituates
Griseofulvin
Sulfa drugs

678
Q

Dont give which antifungal to a patient with AIP?

A

Griseofulvin

679
Q

Antibodies in antiphospholipid syndrome

A

Anti-b2-glycoproteins
Lupus anticoagulant
Anti-cardiolipin

680
Q

Antibodies found in Sneddons syndrome

A

Same as found in anti-phospholipid syndrome (anti-b2-glycoprotein, anti-cardiolipin)
*Snedden syndrome is like the worse form of anti-phospholipid syndrome, where you get multiple ischemic strokes, persistent livedo reticularis, livedo racemosa , labeled hypertension

681
Q

Sneddons syndrome

A

The worse form of anti-phospholipid syndrome (all the same antibodies)

  • livedo racemosa
  • persistent livedo reticularis
  • labile HTN
  • recurrent ischemic strokes
682
Q

What syndrome(s) is b2-glycoprotein found in?

A

Anti-phospholipid syndrome/ Sneddons syndrome

683
Q

What do you treat atrophe Blanche leg lesions with in lupus associated anti-phospholipid syndrome?

A

Anti-malarial

684
Q

Main presenting symptom in cholesterol emboli/warfarin blue toes syndrome

A

Livedo reticularis

685
Q

How long after starting heparin do you see HIT (heparin induced thrombocytopenia)?

A

5-10 days

686
Q

Gene mutation seen in erythromelalgia?

A

JAK-2

687
Q

Treatment for erythromelalgia associated with JAK-2 mutation

A

Anagrelide or aspirin

688
Q

Gene defect associated with paroxysmal nocturnal hemiglobinuria

A

PIG-A gene

689
Q

Treatment for paroxysmal nocturnal hemiglobinuria

A

Eculizumab

690
Q

Gene defect in TTP/HUS

A

ADAMTS13

691
Q

Most common cause of death in Degos disease?

A

GI BLEED

692
Q

NSF (Nephrogenic systemic fibrosis) is associated with what chronic condition?

A

ESRD, a/w exposure to gadolinium-based contrast

693
Q

Which condition is a/w gadolinium-based contrast in patients with ESRD?

A

NSF

694
Q

A patient with chronic kidney disease develops new onset hypertension, woody texture to skin, joint contractures, and tightness of the skin on the hands and the lower legs. What could this be?

A

NSF (nephrogenic systemic fibrosis/nephrogenic systemic sclerosis/nephrogenic fibrosing dermopathy)

695
Q

What type of antibodies can NSF be associated with?

A

Anti-phospholipid antibodies

696
Q

Anti-matrilin-1 protein

A

Relapsing polychondritis

697
Q

Cancer a/w pityriasis rotunda

A

Hepatocellular carcinoma

698
Q

Epidermal nevus could possibly be a mosaic form of what disease?

A

EHK

699
Q 
Type of amyloid in:
Primary systemic amyloid
Secondary systemic amyloid
Hemodialysis associated amyloid
Familial Amyloid
Senile systemic amyloid
A

Primary systemic: AL light chain
Secondary: AA (amyloid associated protein, not immuno)
Hemodialysis associated: b2-microglobulin
Familial amyloid: ATTR (transthyretin)
Senile systemic amyloid (transthyretin)

700
Q

Type of amyloid seen in primary systemic amyloidosis

A

AL (light chain)

701
Q

Type of amyloid see in secondary systemic amyloidosis

A

AA (amyloid associated). This is the type of amyloid associated with systemic disease, like chronic RA, periodic fever syndromes and things like that

702
Q

Type of amyloid seen in hemodialysis associated amyloid

A

B2-microglobulin

703
Q

Type of amyloid seen in both familial amyloid and senile amyloid?

A

ATTR (transthyretin)

704
Q

What is the shoulder pad sign?

A

Amyloid deposits around the periarticular soft tissue. Seen in primary amyloidosis

705
Q

Why do ACE-I give you autoimmune urticaria?

A

Because they cause unregulated generation of bradykinin

706
Q

Intertrigenous plane xanthomas are pathognomonic for which familial hyperlipidemia?

A

Homozygous familial hypercholesterolemia (Type II)

707
Q

For paraneoplastic pemphigus:
Path resembles -
Target antigen -
Common cause of death-

A

Path resembles - EM
Target antigen - plains
Common cause of death- BOOP (bronchiolitis obliterans)

708
Q

Common cause of death in paraneoplastic pemphigus

A

BOOP - bronchiolitis obliterans

709
Q

Which autoimmune disorder is a/w hypergammaglobulinemic purpura of Waldenstrom?

A

Sjogren S

710
Q

What oral endothelium receptor agonist may prevent new ulcers in systemic sclerosis?

A

Bosentan

711
Q

EED is associated with what disease?

A

HIV

712
Q

What treatment worsens peripheral neuropathy in cryoglobulinemia type II/III?

A

Interferon

713
Q

Which cryoglobulinemias are associated with a peripheral neuropathy?

A

Type II/III

714
Q

Permanent livedo reticularis a/w systemic arterial thrombi, labile HTN, neuro sxs

A

Sneddons syndrome

715
Q

Eosinophilic pustular folliculitis (Ofujis) first line treatment:

A

Indomethacin

716
Q

Magenta colored tongue

A

Riboflavin deficiency B2. *also get angular chelitis

717
Q

AFX is a more superficial version of what tumor?

A

MFH (Malignant fibrous histiocytoma)

718
Q

P. Acnes naturally produces what porphyrin?

A

Coproporphyrin III

719
Q

What is the target of light based acne therapy?

A

Coprophyrin III p. 78 Jain

720
Q

Where are the androgen receptors located in the epidermis?

A
  1. Basal layer of the sebaceous glands

2. ORS of the hair follicle

721
Q

Which androgen is produced by which organ?

  1. Testosterone
  2. DHEAS
  3. DHT
A
  1. Testosterone - produced in the gonads
  2. DHEAS - weak androgen produced in the adrenal glands
  3. DHT - not produced anywhere! Testosterone is converted into DHT by 5-alpha reductase in the actual tissues themselves, like the hair follicles, the seminal vessicles, the skin, the penis
    * circulating DHT is 1/20th of circulating testosterone
722
Q

How does taking OCPs help PCOS?

A

It increases SHBG (sex hormone binding globulin), and decreases the amount of free testosterone

*fyi spironolactone blocks the androgen receptor

723
Q

Most well known chemical causing industrial acne? (This is found as an irritant in agent orange)

A

2,3,7,8 tetrachlorobenzodioxin (most common chemical to cause chloracne)

724
Q

Acne found on the malar cheeks, retroauricular, and scrotum

A

Chloracne

725
Q

Name a common EDGFR that causes acne

A

Cetuximab

726
Q

Autosomal dominant syndrome in which you get resistant acne and recurrent bone lesions

A

Apert syndrome

727
Q

What is Apert syndrome?

A

AD- get bone defects and resistant acne

FGFR2

728
Q

In what syndrome do you get pyogenic arthritis, acne, and PG?

A

PAPA syndrome (pyogenic arthritis, PG, acne)

*mutation in CD2BP1 aka PSTPIP1

729
Q

What is another name for Apert syndrome?

A

Acrocephalosyndactyly

730
Q

Another name for acrecephalosyndactyly

A

Apert syndrome (resistant acne plus bone lesions)

731
Q

Polymorphisms in which TLR may be protective against acne fulminans?

A

TLR-4

732
Q

Gene mutation in Apert Syndrome

A

FGFR2

733
Q

Genoderm wtih rosacea-like eruption and verrucous lesions like SKs

A

Haber’s syndrome

734
Q

What is Haber’s syndrome?

A

Genoderm with rosacea-like eruption and verrucous lesions like SKs

735
Q

What cytokines are downregulated in the treatment of rosacea with azelaic acid?

A

Kallikrenin 5

*and also Cathelicidin!

736
Q

Kallikrenin5

A

Cytokine that is downregulated with azelaic acid treatment for rosacea

737
Q

What percentage of patients with rosacea have ocular rosacea?

A

50%!

738
Q

Name some gram negative organisms that commonly occur in gram negative folliculitis

A

Enterobacter, Proteus, Klebsiella, E. Coli

739
Q

Treatment of choice for Ofujis disease

A

Indomethacin

*Ofujis is eosinophilic pustular folliculitis, occurs in 30 year old Japanese men

740
Q

What are the characteristics of the nerves that control sweating?

A

They are anatomically sympathetic but function as cholinergic

741
Q

Freys syndrome is caused by disruption of what nerve?

A

Auriculotemporal nerve (of V1)

742
Q

What chromosomal region is present in over 50% of Psoriasis patients and contains the cw6-allele? Also what chromasome is it on?

A

The PSORS1 (psoriasis susecptibility region)! Which is located on chromasome 6p

743
Q

What chromosome is PSORS1 located on?

A

Chromosome 6p

744
Q

What important gene does the PSORS1 allele contain?

A

Cw-6

745
Q

Which psoriasis allele is most strongly associated with early onset and guttate psoriasis?

A

Cw-6

746
Q

What two types of psoriasis is HLA-cw6 assciated with?

A
  1. Early onset
  2. Guttate

*and actually its found in over 50% of adult onset PSO patients as well

747
Q

HLA B27 is associated with what three type of psoriasis?

A

Psoriatic arthritis
Sarco-ilitis-associated Psoriasis
Pustular psoriasis

748
Q

Which allele is most strongly associated with:

  1. Pustular psoriasis
  2. Guttate psoriasis
A
  1. HLA -B27

2. HLA- cw6 (and actually B13/B17 as well)

749
Q

Which cytokine upregulation in psoriasis is responsible for increased keritinocyte proliferation?

A

STAT-3

750
Q

What two medications induce psoriasis quickly (<4 week period)?

A

Terbinafine, NSAIDS

751
Q

What two medications induce psoriasis in an intermediate time frame? (4-12 week period?)

A

ACE inhibitors, antimalarials

752
Q

What two medications induce psoriasis in a long term period? (>12 weeks)

A

Beta-blockers, lithium

753
Q

What percentage of guttate pso patients progress to plaque type?

A

40%

754
Q

What is the recommended treatment for impetigo herpetiformis

A

EARLY DELIVERY

755
Q

What acne syndrome can palmoplantar-plantar pustulosis be associated with?

A

SAPHO

756
Q

Number 1 cause of pityriasis amiantacea

A

PSORIASIS

757
Q

What systemic do you use as monotherapy for erythrodermic and pustular psoriasis?

A

Acitretin

758
Q

Which psoriasis variants do you use Acitretin for?

A

Pustular Psoriasis

Erythrodermic psoriasis

759
Q

Which psoriasis medication should you not use in combination with salicylic acid?

A

Vitamin D analogs like Dovonex (calciptriol)

760
Q

Which psoriasis medications should you not use right before NBUVB because it causes tumors?

A

Vitamin D analogs like dovonex (calcipotriol)

761
Q

Name a couple of risk factors for pustular psoriasis

A

Hypocalcemia

Pregnancy (impetigo herpetiformis)

762
Q

What lab will be decreased in a patient with pustular psoriasis?

A

Hypoalbunemia

763
Q

Treatment of choice for pustular psoriasis

A

Cyclosporine

764
Q

HLA type for pustular psoriasis

A

HLA-B27

765
Q

Treatment of choice for palmoplantar pustulosis

A

Acitretin

766
Q

Name a psoriasis medication that commonly causes gingival hyperplasia

A

Cyclosporine

767
Q

Name some side effects of cyclosporine

A 
HTN
Nephrotoxicity
Gingival hyperplasia
HYPERkalemia/hypomagnesemia
Gout
768
Q

Most common childhood type of PRP

A

Type IV! (Type III is the type that is most like the adult form, type I, but actually type 4 is the most common in children and just presents with plaques on the hands and knees not generalized)

769
Q

Characteristic of Type V PRP

A

Sclerodermoid changes

770
Q

Characteristics of Type III PRP

A

Childhood PRP, similar to adult onset PRP. Not the most come (type IV is for kids)

771
Q

What is the most common location for annular LP?

A

Penis

772
Q

How long is the offending medication usually taken before drug-induced LP is seen?

A

Several months

773
Q

Which form of LP carries a slight risk of SCC with longstanding lesions

A

Erosive or ulcerated LP (found in the oral mucosa and palmoplantar surface) of

774
Q

Nail findings is nail LP

A

Dorsal pterygium
20+ nail dystrophy
Lateral thinning
Longitudinal ridging

775
Q

Findings in Graham-Little-Piccardi-Lasseur syndrome

A

Type of lichoplanopilaris (LPP)=

Typical LP+scarring alopecia of the scalp+ nonscarring loss of axillary/pubic hair

776
Q

Scarring alopecia, type of LP

A

Lichen planopilaris

777
Q

Specific type of LPP, triad of follicular LP plus scarring alopecia of the scalp and nonscarring alopecia of the genitals

A

Graham Little Piccardi syndrome

778
Q

What percentage of chronic GVHD patients arise from acute GVHD vs de novo?

A

It’s about 50/50!

779
Q

What is the main cause of death in chronic GVHD?

A

Infection due to immunosuppression

780
Q

Most common type of solid organ transplant to cause GVHD?

A

SMALL INTESTINE!!!

781
Q

Single most important predictor of developing GVHD is what?

A

HLA compatibility

782
Q

Which type of stem cell blood products carry the highest risk for developing GVHD? (Peripheral blood vs cord blood vs bone marrow)

A

Peripheral blood > bone marrow > cord blood

783
Q

What type of blood treatments for stem cell transplants increase the risk for “delayed onset acute GVHD”

A

Non-myeloablative regimens

784
Q

What is the most commonly affected organ in all GVHD reactions?

A

SKIN!

785
Q

What monoclonal antibody is helpful in some cases of chronic GVHD?

A

Rituximab

786
Q

What are two clinical findings which are very characteristic of acute GVHD?

A

Acral erythema

Violaceous hue on the ear

787
Q

What other organs are commonly affected in addition to skin in acute GVHD?

A

GI tract and liver

788
Q

How is liver severity assessed in acute GVHD?

A

By degree of bilirubin elevation

*extent of GI involvement evaluated by the amount of DIARRHEA

789
Q

What drug has been shown to decrease the incidence of visceral GVHD?

A

Maraviroc (CCR5 inhibitor)

*note that it decreases visceral involvement like GI and liver but NOT anything in the skin

790
Q

What cytokine does Maraviroc inhibit?

A

Maraviroc is a CCR5 inhibitor. It decreases the incidence of visceral (not skin GVHD)

791
Q

What CCR5 inhibitor decreases the incidence of visceral GVHD?

A

Maraviroc

792
Q

1 site for LP

A

Oral/mucosal

793
Q

What percentage of patients with oral LP will develop skin LP?

A

Only 10%!

794
Q

Shulmans Syndrome

A

Eosinophilic Fascitis

795
Q

What types of cells does LP lack?

A

Eosinophils and parakaratosis! (With the exception of drug induced LP and oral LP

796
Q

Treatment of choice for Erythema Dyschromicum perstans

A

Clofazamine! (Weird. Usually for treating leprosy)

797
Q

Where does impetigo herpetiformis start usually?

A

In the groin! - then spreads to flexures

798
Q

Risk to fetus with impetigo herpetiformis

A

Placental insufficiency
Stillbirth
Neonatal death
A/w hypocalcemia

799
Q

Does impetigo herpetiformis occur again with subsequent pregnancies?

A

YES and also OCPs

800
Q

What trimester does impetigo herpetiformis usually occur in?

A

Third trimester

801
Q

Which trimester does prurigo of pregnancy occur in?

A

First *most of the other dermatosis occur in the third

802
Q

Treatment for Intrahepatic cholestasis of pregnancy? And what does this do?

A

Oral ursodeoxycholic acid - this decreases serum bile acid levels

803
Q

Risk to fetus with intrahepatic chol. Of pregnancy

A

Prematurity
Stillbirth
Fetal distress

804
Q

Risk to fetus in PUPP

A

NONE!!!

805
Q

Which trimester does PUPP usually show up?

A

THIRD

806
Q

Treatment for PUPP

A

Topical steroids

807
Q

Which pregnancy does PUPP occur in usually

A

The first. Does not usually recur with subsequent pregnancies

808
Q

Risk factor for PUPP

A

Multiple gestational pregnancies

809
Q

Risk to fetus with pemphigoid gestationis

A

Prematurity and SGA

810
Q

What non-pregnancy diagnosis can pemphigoid gestationis occur in?

A

Choriocarcinoma

811
Q

What disease are patients with pemphigoid gestationis at risk for post partum?

A

Graves

812
Q

Which HLA types are pemphigoid gestationis patients strongly associated with?

A

HLA DR3-DR4

813
Q

Which pregnancy dermatosis does not usually recur with subsequent pregnancies?

A

PUPP is the only one.

814
Q

Which pregnancy diagnosis spares the umbilicus and which one involves it?

A

PUPP spares the umbilicus and pemphigus gestationis involves it

815
Q

Side effect of Bexarotene

A

Reversible hypothyroidism

816
Q

Which retinoid causes a side effect of reversible hypothyroidism

A

Bexarotene

817
Q

What sort of thyroid dysfunction is Bexarotene associated with?

A

Reversible HYPOthyroidism

818
Q

What kind of drug reaction does azathioprine cause?

A

SCLE-like reaction! It’s known for this

819
Q

another name for Von Hebras disease?

A

EM minor

820
Q

How many weeks prior does herpes labialis usually precede EM?

A

3 weeks

821
Q

Cases of EM caused by Histoplasma capsulatum usually also have what other concomitant condition happening at the same time?

A

Erythema nodosum

822
Q

Erythema Nodosum plus EM together - which type of infection should you be suspicious of?

A

Histoplasma Capsulatum

823
Q

Most common cause of EM major

A

Mycoplasma >HSV (Hocker)

824
Q

What is a useful clinical tool to distinguish EM from SJS/TEN?

A

EM will be PAPULAR and elevated, distributed in the head and neck, and then the acral areas

VS

SJS/TEN will always be more macular

825
Q

HLA type of Asian/East Indian people with high risk for SJS/TEN due to carbamazepine

A

HLA-B1502

*HLA B3101 is Europeans exposed to carbamazipine

HLA-B5801 Han Chinese exposed to allopurinol

826
Q

Which drug taken by patients of East Indian/Asian decent increases their risk for SJS/TEN, and what is their HLA type?

A

Carbamazepine

HLA -B1501

*HLA 3101 is Europeans taking carbamazipine

827
Q

HLA type in Han Chinese exposed to allopurinol with increased risk for SJS/TEN

A

HLA-B5801’

828
Q

What is the main cellular mediator of apoptosis in SJS/TEN?

A

Granulyisin

829
Q

Granulysin

A

The main mediator of apoptosis in SJS/TEN

830
Q

Which cellular mediator in SJS/TEN leads to activation of caspases and then eventually apoptosis?

A

The binding of Fas/FasL

831
Q

In regards to SJS/TEN, Which anticonvulsant does NOT cross react with other medications in its class?

A

Valproic acid

*Lamotrigine does not cross react with aromatic anticonvulsants

832
Q

T/F you can get respiratory involvement in SJS/TEN?

A

TRUE - in fact in 25%

833
Q

Where do SJS/TEN lesions typically start on the body?

A

On the trunk! (Vs EM usually on the face and acral)

834
Q

What is the only vital sign in the SCORTEN criteria (SJS/TEN)

A

HR

SCORTEN
Age (>40yrs)
Associated malignancy
HR
BUN
Glucose
Serum bicarbonate
>10% BSA
835
Q

What are the only 3 lab values in the SCORTEN criteria?

A

Serum BUN
Serum bicarb
Glucose

*TAMEBUG
Tachycardia
Age
Malignancy
Epidermal loss
Bicarb
Urea 
Glucose
836
Q

How many points on the SCORTEN criteria give you a >90% mortality rate for SJS/TEN?

A

5

837
Q

Which point of the SCORTEN criteria is the single most important risk factor for mortality?

A

Serum BICARB

838
Q

FDA recommends screening for which HLA type before giving abacavir?

A

HLA-B5701 *HIGH RISK FOR SJS/TEN!!!!

839
Q

HLA-B1507 a/w high risk of what disease?

A

SJS/TEN after giving ABACAVIR!

840
Q

Which drug carries a high incidence of SJS/TEN in patients who are HLA-B1507 positive?

A

ABACAVIR!

841
Q

Avoid drugs with long half-lives or short half lives in patients with SJS/TEN?

A

Avoid drugs with long half lives!!!

842
Q

Most common SJS/TEN complication?

A

Ocular sequelae

843
Q

Most common cause of death in SJS/TEN

A

Infections (pseudomonas or s aureus)

844
Q

T/F sulfa antibiotics do not cross-react with non-antibiotic sulfonamides like HCTZ and hypoglycemic agents?

A

TRUE they do not

845
Q

What is the severe variant of PLEVA called which has skin lesions plus fever, lymphadenopathy, Mucocutaneous, pulmonary, GI involvement?

A

Febrile ulceronecrotic Mucha-Haberman disease

846
Q

Does PLC resolve with hypopigmentation or hyperpigmentation notoriously?

A

HYPOpigmentation

847
Q

Both PLEVA and PLC have a strict absence of what type of cell?

A

Eosinophils!!!!!

848
Q

What type of cell predominates in PLEVA? (That helps distinguish it from other diseases)

A

CD8 positive lymphocytes

*also remember PLEVA has an absence of eos

849
Q

Most common FDE:

  • overall
  • nonpigmented
  • children
  • penis
A
  • overall: Tetracyclines (although Hocker says sulfa is most common overall not TCN
  • nonpigmented:pseudoepedrine
  • children: bactrim
  • penis: sulfa
850
Q

What body site does naproxen FDE have a predilection for?

A

The lips!

851
Q

Which FDE causing medication has a predilection for the lips?

A

Naproxen!

852
Q

What chemical which was previously found in laxatives has now been removed due to common occurrences of fixed drug eruption?

A

Phenolphthalein

853
Q

1 body site for fixed drug eruption?

A

Oral and genital mucosa!

854
Q

Adult linear IgA-bullous dermatosis a/w what drug?

A

VANCOMYCIN!

855
Q

Blue grey pigment in the cartilage

A

Alkaptonuria

856
Q

Blue-grey pigmentation on the face, ears/catilage, and on the sclera - name the mutation?

And what are the complications

A

This is alkaptonuria - homogentisic 1,2,-deoxygenase gene (HGO)

Lots of arthroscopic surgeries, risk of MI due to aortic/mitral valvulitis

857
Q

Three primary organisms to cause nail

A

Scydillium
Saprophiatsis
Candida

858
Q

The ice cube test is positive in which of these cold urticaria Primary cold contact urticaria
Secondary cold contact urticaria
Reflex cold urticaria
Familial cold urticaria

A

ONLY in primary cold contact urticaria

859
Q

Aquagenic urticaria is more common in what patient population

A

Cystic fibrosis patients

860
Q

What type of drugs are first line for urticarial vasculitis?

A

NSAIDS

861
Q

Syndrome of chronic urticaria, fevers, bone pain, arthralgias, increased ESR and an IgM gammopathy

A

Schnitzler’s syndrome. “SchMitzlers syndrome”

862
Q

Name the findings of Schnitzlers syndrome

A 
Chronic urticaria
Chronic urticaria
Fevers
Bone pain
Elevated ESR
IgM gammopathy

*treat with anakinra

863
Q

What do you treat Schnitzlers syndrome with?

A

Anakinra

*remember Schnitzlers is chronic urticaria plus bone pain, fevers, IgM gammopathy

864
Q

What type of monoclonal gammopathy do you get in Schnitzlers syndrome?

A

IgM! “SchMitzlers syndrome”!!!!

865
Q

Hypocomplementemic urticarial vasculitis will have what notably on labs?

A

Low complement levels, anti-C1q antibody

866
Q

What inflammatory marker is the cause of all angioedema without urticaria (HAE, AAE, and ACE-I induced angioedema)

A

Excess bradykinin

867
Q

What deficiency must you rule out when evaluating a case of angioedema without urticaria?

A

C1 esterase inhibitor deficiency

868
Q

What is the screening test of choice for hereditary and aquired angioedema?

A

C4

869
Q

What is used for regular prophylactic treatment for hereditary angioedema, and what is used during acute attacks?

A

Danazol or Stanazol for ppx, and C1H during acute attacks

870
Q

Which type of angioedema without urticaria is characterized by increased significant facial swelling?

A

Type III! (which is aquired angioedema usually due to lymphoproliferative disorders

871
Q

What labs should you check if you suspect angioedema without urticaria

A

C1H, C1q, C4

872
Q

Erythema marginatum happens in kids who were not treated for what kind of infection?

A

Group A beta-hemolytic strep (seen in the setting of rheumatic fever)

873
Q

Treatment of choice for EED

A

Dapsone

874
Q

EED is a/w what kind of monoclonal gammopathy

A

IgA monoclonal gammopathy

875
Q

Poor man’s test for cryoglobulinemia

A

RF!

876
Q

If you see Raynauds of the ears, what is this a clinical clue for?

A

CRYOGLOBULINEMIA!

877
Q

What are the two ELISA tests that you use for detecting wegeners/Churg Strauss?

A

MPO - an ELISA that confirms P-ANCA in Churgg Strauss

PR3 - an ELISA that confirms C-ANCA in Wegeners

878
Q

What part of the vascular complex is the immune deposition located in cutaneous small vessel vasculitis?

A

The post capillary venules

879
Q

What street drug can cause CSVV

A

Levamisole-laced cocaine

880
Q

What is a common and okay finding to see on DIF when you biopsy CSVV

A

80% w/perivascular C3 and IgM

881
Q

What is a key predictor of nephritis in children with HSP?

A

Abdominal pain

882
Q

Urticarial vasculitis is a/w which systemic disease

EED vasculitis is a/w which systemic disease?

A

Urticarial vasculitis - SLE

EED- HIV

883
Q

Which disease has renal involvement and which doesnt generally?

Wegeners
Vs
Churg Strauss

A

Wegeners does!

Not Churg Strauss

884
Q

Which is granulomatous: Wegeners or Churg Strauss?

A

Wegeners! Not Churg Strauss!!

885
Q

Perinuclear staining vs Granular cytoplasmic staining:

Wegeners vs Churg Strauss?

A

Perinuclear is Churg Strauss (P-ANCA/MPO)
Granular Cytoplasmic is Wegeners (C-ANCA/PR3)

*maybe think bc Wegeners causes granulomas? “Granular cytoplasmic”

886
Q

If you do not treat Wegeners, within one year what will they die from???

A

Renal disease!!! 80% 1 year mortality

887
Q

What clinical sign is pathopneumonic for Wegeners?

A

Strawberry gums

888
Q

Most common cause of pulmonary renal syndrome

A

MPA (microscopic polyangitis)

889
Q

Omalizumab (Xolair) can actually induce which vasculitis?

A

Churg Strauss

890
Q

What drug induced lupus erythematosus with a positive P-ANCA?

A

Minocycline-induced lupus!

*technically also levamisole as well

891
Q

Which ANCA vaculidity creates a Th1 response?

A

C-ANCA (Wegeners) this is why you get granuloma formation in wegeners bc of the Th1 response

892
Q

Which ANCA vasculidity is completely without granuloma formation?

A

MPA

893
Q

Two key clinical features of Churg Strauss

A

Asthma and Eosinophilia

894
Q

What is usually the first clinical presentation of Churg Strauss?

A

Adult onset asthma
Nasal polyps
Allergic rhinitis

*then second stage later on comes eosinophilia and then the vasculitis in the third stage

895
Q

Name two diseases that PAN (polyarteritis nodosa) is associated with

A

Hep B
Hairy cell leukemia

*sidenote has also be a/w strep in children, and also a/w minocycline!

896
Q

What is the most commonly positive antibody in anti-phospholipid syndrome?

And how abt the most specific?

A

Anti-cardiolipin is most common

Anti-B2-glycoprotein is the most specific

897
Q

Anti-cardiolipin antibodies

A

Most common positive in APLS

*other common Ab are lupus anticoagulant, anti-b2-glycoprotein

898
Q

What drug is known for causing cause livedo reticularis?

A

Amantidine

899
Q

Most common complication from parotidectomy

A

Auriculotemporal (Frey syndrome)

900
Q

Mucha-Haberman disease

A

“Febrile ulceronecrotic Mucha-Haberman disease” is a really severe form of PLEVA that occurs with high fever, lymphadenopathy, arthritis, mucosa stuff, GI
*high TNF alpha levels

901
Q

What is the severe form of PLEVA called?

A

Febrile ulceronecrotic Mucha-Haberman disease

902
Q

Which type of interface dermatitis has no eosinophils

A

PLEVA and PLC

903
Q

What is the best predictor for speed of disease resolution

A

Diffuse distribution

904
Q

Most common auto immune disease a/w lichen sclerosis

A

Autoimmune thyroid

905
Q

Most people with lichen sclerosis have circulating antibodies to what protein?

A

ECM-1.

*ECM-1 also seen in lipoid proteinosis

906
Q

50% of all vulvar SCCs occur in the setting of what disease?

A

LS&A!

907
Q

What is Schnitzlers syndrome?

A

Urticarial vasculitis plus monoclonal IgM plus systemic features like fever/bone pain/ect

908
Q

Name of the syndrome: urticarial vasculitis plus IgM antibody plus fever/bone pain

A

Schnitzlers syndrome (SchMitzlers syndrome)

909
Q

What type of Monoclonal Ab seen in Schnitzlers syndrome?

A

Monoclonal IgM

910
Q

Which type of lupus carries a risk of SCC?

A

Hypertrophic Lupus (basically hypertrophic LP on the upper half of the body)

911
Q

What three reasons could you have for getting carotenemia?

A
  1. Eating too many carrots
  2. Hypothyroid
  3. DM

*bc cant convert the vitamin

912
Q

Name a common practice that is a risk factor for DLE

A

Smoking!

913
Q

What percent of SLE patients will have DLE?

And what percent of DLE patient will progress to SLE?

A

40% of SLE patients will have DLE lesions
But only 5-20% of DLE patients will progress to SLE

  • fyi 5% risk factor if DLE only on your head
  • 20% risk factor if DLE is diffuse
914
Q

What percent of DLE patients have mucosal involvement>?

A

25%

915
Q

ANA is positive in what percent of DLE patients?

A

Only 5-25%

916
Q

Which type of chronic lupus has an increased risk of SCC?

A

Hypertrophic LE

917
Q

What is the difference in clinical distribution of hypertrophic LP vs hypertrophic LE? (lupus erythematosus)

A

Hypertrophic LE favors the UPPER HALF of the body while hypertrophic LP favors the LOWER HALF of the body

918
Q

What is reticular erythematous mucinosis

A

Histologically similar to Jessners, but is morphological distinctive : erythematous macules and papules on the mid back/chest in a reticular pattern)

919
Q

Treatment for tumid lupus

A

Tumid lupus is usually a solitary indurated plaque on the face. Treatment includes antimalarials

920
Q

Most specific antibody for Sjögren’s syndrome

A

Alpha-fodrin

921
Q

Which is more likely to progress to SLE: childhood DLE or adult DLE?

A

Childhood DLE and widespread DLE have a greater chance of progressing to SLE (20%)

922
Q

How do you tell the difference between Jessners and tumid lupus?

A

Jessners is a pseudolymphoma and so it has a CD8 infiltrate and no mucin

Tumid lupus has a ton of mucin and no CD8

923
Q

What would you choose to treat lupus if topical treatments aren’t working?

A

First - hydroxychloroquine

Second, can add quinacrine

924
Q

What is the most common systemic finding in SCLE?

A

Arthralgias (70%)

925
Q

Most common antibody found in SCLE

A

Anti-Ro/SSA

926
Q

Which kind of SCLE is it rare to have systemic SLE?

A

Drug induced SCLE has no systemic involvement usually, just skin

927
Q

Name the most common drug that causes drug induced SCLE

A

HCTZ

*other common ones are griseofulvin, terbinafine, calcium channel blockers, eat receipt

928
Q

Which antibody most commonly found in 99% of neonatal lupus?

A

Anti-Ro/SSA

929
Q

What percent of women undiagnosed SLE women who have Ro Ab will have a child with neonatal lupus?

  • How abt women wtih known SLE and Ro ab
  • How abt women w ho have had a prior child with neonatal lupus?
A
  • 1% chance if undiagnosed
  • 15% chance if have lupus
  • 25% chance of having a second child with neonatal lupus if you’ve already had one
930
Q

Alternative treatment for LPP

A

Pioglitazone

931
Q

Drug association with multiple osteoma cutis on a middle aged lady’s face

A

Tetracyclines (just think about acne and acne treatments)

932
Q

Habers syndrome

A

Genoderm with Rosacea and lots of SKs on the arms (some people believe it just may be Dowling Degos disease)

933
Q

Dowling Degos disease a/w what genoderm?

A

EBS with mottled pigmentation (Dowling degos is reticulated hyperpigmentation adult onset, in the axilla and groin)

AD mutation in Keratin 5

934
Q

What layer of the skin is keratin 5 found in, and what two diseases (one is congenital and one is adult onset) are a/w this mutation?

A

K 5/14 = BASAL layer!

EBS with mottled pigmentation &
Dowling-Degos disease!

K5 is a/w reticulated hyperpigmentation

935
Q

A patient presents with dystrophic nails, PPK, natal teeth, and a steatocystoma. What disorder and what mutation?

A

Pachyonychia Congenita Type II (Keratin 6/17)= PPK, nail dystrophy, steatocystomas, natal teeth

*Pach congenita Type I is Keratin 6a/16 = PPK, nail dystrophy, and benign leukoplakia

936
Q

Polymorphisms in which TLR are protective against acne fulminans?

A

TLR-4

937
Q

Name the HLA classes a/w guttate psoriasis

A

HLA Cw6, B13/17

HLA Cw6 - early onset, guttate
HLA B27 - pustular psoriasis, psoriatic arthritis
HLA B13/17 - erythroderma and guttate psoriasis

938
Q

HLA class a/w early onset psoriasis

A

HLA Cw6

HLA Cw6 - early onset, guttate
HLA B27 - pustular psoriasis, psoriatic arthritis
HLA B13/17 - erythroderma and guttate psoriasis

939
Q

HLA class a/w erythrodermic psoriasis

A

HLA Cw6, B13/17

HLA Cw6 - early onset, guttate
HLA B27 - pustular psoriasis, psoriatic arthritis
HLA B13/17 - erythroderma and guttate psoriasis

940
Q

Name the two types of psoriasis neutrophil collections in the epidermis

A

Munro microabcesses - collections of neuts in the stratum corneum
Spongiform pustules of Kogoj - neuts in spinosum

941
Q

Munro microabcesses

A

Collections of neuts in corneum. Found in PSORIASIS

*spong pustules of Kogoj - neuts in the spinous layer!

942
Q

Treatment of choice for pustular psoriasis

A

Cyclosporine

943
Q

What two questions do you ask before starting cyclosporine?

A

HTN?

Kidney probs?

944
Q

Risk factors for pustular psoriasis?

A

Stopping a systemic corticosteroid
Hypocalcemia
Pregnancy (impetigo herpetiformis)

945
Q

Person presents with tense sterile pustules on their hands and feet - what other physical exam symptom should you ask abt?

A

Any sternoclavicular tenderness? (Can be associated with SAPHO syndrome)

946
Q

Side effects of cyclosporine

A 
HTN, kidney probs (Duh)
GINGIVAL HYPERPLASIA 
HYPERKalemia
HYPOmag
INCREASED uric acid (why you ask abt gout)
Hypertrichosis
947
Q

Name some things that cyclosporine is used to treat

A

Pustular psoriasis
PG
Severe atopic derm
Autoimmune bullous disorders

948
Q

A person with severe pustular psoriasis is being treated with a systemic agent and his gums begin to become larger - what med is it?

A

Cyclosporine ( causes GINGIVAL HYPERPLASIA)

949
Q

If a person’s creatinine rises above what percent of baseline, you should reduce the dose of cyclosporine

A

30%!

950
Q

How long is it safe to give cyclosporine for?

A

1 year and no more

951
Q

What is the mechanism of action of cyclosporine?

A

Inhibits T cells by binding to cyclophillin (blocks activation of calcineurin activation which does NFAT, so it decreases IL-2

952
Q

Child sarcoid

A

Blau Syndrome

953
Q

Blau syndrome

A

Child sarcoid

954
Q

Acute sarcoid

A

Lofgrens Syndrome (EN, hilar adenopathy, acute iritis, migrating polyarthritis)

955
Q

Sarcoid with sicca symptoms

A

Miculitz syndrome

956
Q

Panniculitis due to sarcoid

A

Darier-Roussey Syndrome

957
Q

Darier-Roussey syndrome

A

Sarcoid panniculitis

958
Q

“Uveoparotid fever”

A

Heerfordts syndrome (Parotid glad + uveitis +facial nerve palsy)

959
Q

What type of uveitis is characteristic of sarcoid?

A

Anterior uveitis

960
Q

Hilar adenopathy on X-ray
E Nodosum on shins
Migratory polyarthritis of ankles

A

Lofgrens syndrome (Acute sarcoid)

961
Q

Large painful subq nodules on the arms and legs, this is a variant of sarcoid

A

Darier Roussey - sarcoid panniculitis

962
Q

Uveitis
Parotiditis
Facial palsy

A

Heerfordts syndrome - variant of sarcoid

963
Q

Enlargement of lacrimal gland
Enlargement of Parotid glad
Sicca symptoms

A

Miculitz Syndrome - variant of sarcoid (and can be seen in Sjogrens)

964
Q

Inflamed eye
Ipsilateral lymphadenopathy
Fever

A

Parinaud ocular glandular syndrome (variant of sarcoid)

*also seen in tularemia and catscratch disease

965
Q

What diseases is Parinaud ocular glandular syndrome seen in?

A

SARCOID variant
Tularemia
Cat scratch disease

966
Q

Old school sarcoid test:

A

Kviem -Siltzback test

967
Q

Kveim-Siltzback test

A

Old school sarcoid test

968
Q

What type of collagen is overexpressed in keloids

A

Collagen III

969
Q

Gene defect in Muckle Wells

A

CIAS1 (“fuckle Wells” with CIAlis)

970
Q

A patient gets chronic cold urticaria, deafness, renal amyloidosis, attacks of fever, abdominal pain, myalgias. What is the diagnosis and gene defect

A

Muckle Wells

Gene defect CIAS, which encodes cryopyrin

971
Q

Gene defect for Familial Mediterranean Fever

A

MEFR which encodes pyrin, also known as marenostrin

“A family of mediterranean pyros goes to Reno”

972
Q

Reccurent episodes of fever, erysipelas like erythema (legs, polyserositis. What is the diagnosis?

And what do you treat with?

A

Familial Mediterranean Fever syndrome

Treat with colchicine
Remember you treat muckle wells with anakinra

973
Q

Treatment for Muckle Wells

A

Anakinra

974
Q

Patient gets high fevers, erythematous annular or serpiginous patches on the extremities, abdominal pain, arthralgias What is the diagnosis and what do you treat with?

A

TRAPS (TNF alpha associated periodic syndrome). TNFR-1 mutation

Treat with TNF alpha inhibitors

975
Q

Another name for Muckle Wells

A

Familial Cold Urticaria

976
Q

Another name for Familial Cold Urticaria Syndrome

A

Muckle Wells

977
Q

AA protein is seen in which type of amyloidosis?

A

Secondary systemic amyloid (usually found in chronic inflammatory diseases like RA, TB, or the periodic fever syndromes like muckle wells, traps, ect

978
Q

Type of amyloid seen in secondary systemic amyloid

A

AA

979
Q

AA (amyloid associated protein) loses its Congo red affinity when exposed to what chemical?

A

Potassium permangonate

980
Q

What do you treat all of the chronic fever syndromes with except familial meditteranean fever?

A

Anakinra!

*and you treat FMF with colchicine

981
Q

Which is the only periodic fever syndrome that you dont treat with anakinra and what do you treat it with instead?

A

Familial Mediterranean Fever syndrome! Treat with colchicine

982
Q

What type of systemic symptoms do you look out for with someone who has scleredema

A

Dysphasia
Cardiac abnormalities
Serositis

983
Q

What are the three types of Scleredema

A

Type I: Strep related
Type II: Monoclonal gammopathy related
Type III: DM related

984
Q

Treatment for Type II, III scleredema?

A

Phototherapy
Cyclophosphamide
Cyclosporine
Steroids

985
Q

What is the localized form of scleromyxedema called?

A

Papular mucinosis (lichen myxedematosus)

986
Q

Diagnosis: erythematous macules and papules in reticulated pattern over the midline chest and back, induced by UV light

A

Reticular erythematous mucinosis

*this overlaps with tumid lupus, due to overactive IL-1
DIF is negative!

987
Q

Several members of a family have been diagnosed with lichen amyloid. What gene mutation should they all be tested for?

A

RET oncogene. They could all have Sipple Syndrome! (MEN2A)

  • medullary thyroid cancer
  • pheochromocytoma
  • hyperparathyroid

*could consider a prophylactic thyroidectomy

988
Q

Chronic seb derm, erythroderma, frequent infections. What disease is this and what is its cause?

A

Leiners disease! Caused by a decrease in C5

989
Q

Fogo selvagem caused by what organism?

A

Black fly (simulium spp.)

*black fly also causes onchocerciasis

990
Q

The black fly causes what blistering disorder?

A

Fogo selvagem (pemphigus foliaceous)

*also causes oncocerciasis

991
Q

Vector for onchocerciasis

A

Black fly (simulium spp.)

*also vector for pemphigus foliaceous -fogo selvagem

992
Q

Mazotti Reaction

A

Severe urticarial reaction after getting diethylcarbamzine when being treated for oncocerciasis (vector is the black fly, simulium spp. Which also causes pemphigus folicacous fogo selvagem)

993
Q

Subcutaneous nodules containing worms,
Depigmentation on the lower legs
Vision loss

A

Oncocerciasis (River blindness)

*caused by black fly (simulium spp. Which also causes fogo selvagem)

994
Q

What do you watch out for when treating river blindness

A

Mazotti reaction. Which is severe urticaria when given diethylcarbizine. River blindness is oncocerciasis, which presents as papules filled wtih worms and hypopigmented lesions on legs. Vector is the black fly (simulium spp) which is the same vector that causes fogo selvagem

995
Q

What two blistering disorders show IIF binding to the dermal side of the BMZ with salt split skin?

A

EBA (Collagen VII) (anhoring fibrils in sublamina densa)
And
Anti-epilligrin (laminin 5) Cicatricial pemphigoid (binds to anchoring filaments on the bottom of lamina lucida

996
Q

What is the name of the syndrome:

Angiosarcoma in a radiated lymphedematous area like after mastectomy

A

Stewart-Treves syndrome

997
Q

Stewart Treves syndrome

A

Angiosarcoma occurring in chronic lymphedema (usually following breast cancer with lymph node dissection)

998
Q

Clinical scenario: a 56 year old lady who is s/p double mastectomy w axillary lymph node dissection and radiation develops ill-defined bruise-like macule that becomes a plaque

A

Stewart Treves syndrome: angiosarc in chronic lymphedema

999
Q

Stain for angiosarc

A

C-myc positive ***BOARDS

1000
Q

Scleromyxedema a/w with which gammopathy?

A

IgG y (lambda)

1001
Q

NSF a/w with exposure to what

A

Gadolinium contrast

1002
Q

A patient is undergoing dialysis and gets a large sclerotic indurated plaque on his body. What is this most likely?

A

Nephrogenic systemic fibrosis (resembles scleroderma clinically but scleromyxedema pathologically)

1003
Q

Donovan bodies

A

Parasitized histiocytes in Granuloma Inguinale

1004
Q

What are the parasitized histiocytes in Granuloma inguinale called?

A

Donovan bodies

1005
Q

DDx for parasitized histiocytes

A 
Leichmaniasis
Histoplasmosis
Granuloma inguinale (Donovan bodies)
Rhinoscleroma (Mikulicz cells, Russell bodies)
Toxoplasmosis
1006
Q

New first line treatment for Hailey Hailey

A

Naltrexone! (*HOT boards fodder right now)

1007
Q

Calciphylaxis a/w with what lab values

A

Increased serum phosphate
Hypercoagulable state
HYPERparathyroidism

1008
Q

30% of patients with granulomatous slack skin will go on to develop what kind of malignancy?

A

Hodgkins

1009
Q

Adult T cell leukemia/lymphoma a/w what virus?

A

HTLV-1 (endemic in africa, Japan, carribean)

*a/w CD25!

1010
Q

Adult T cell leukemia/lymphoma a/w what lab value?

A

Hypercalcemia

*remember this is the lymphoma that is a/w HTLV-1 and the “floret/clover cells”, and is indistinguishable from MF except for these cells

1011
Q

CD25+

A

Adult T cell lymphoma/leukemia (a/w HTLV-1)

1012
Q

Most common cause of death in Churgg Strauss

A

Myocarditis and coronary arthritis! *weird. This is per Hocker

1013
Q

Erythroderma is a/w with what cardiac finding?

A

HIGH OUTPUT CARDIAC FAILURE*weird

1014
Q

What lab finding in 90% of patients with Neuroblastoma?

A

Increased urinary catecholamines (bc remember they get raccoon eyes and the skin Mets welp up when you stroke them - due to release of the catecholamines)

1015
Q

What lab should you test for a diagnosis of neuroblastoma?

A

Urinary catecholamines are increased

1016
Q

2nd most common solid malignancy of childhood

A

Neuroblastoma

1017
Q

Older children have a better or worse prognosis with Neuroblastoma

A

Older children have a WORSE prognosis

1018
Q

What stain will be negative in Merkel cell?

A

TTF-1

Also negative: S100, CK7, CEA

1019
Q

What cellular expression marker gives a worse prognosis in Merkel cell?

A

P63

1020
Q

Skin findings in Reed syndrome

A

Multiple Leiomyomas

1021
Q

Cancer a/w Reed syndrome

A

RCC

1022
Q

Enzyme mutated in Reed syndrome

A

Fumarate hydra tase

1023
Q

What is the difference in origin of a Leiomyoma vs a Angioleiomyoma

A

Leiomyoma is derived from smooth muscle of the arrector pili vs Angioleiomyoma derived from the smooth muscle of the subcutaneous blood vessels

1024
Q

What is the most potent anaphylatoxin?

A

C5a

DERMATOLOGY (16 decks)

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