Gen Derm 4 Flashcards
1
Q
spontaneous multiple keloids
A
noonan syndrome
2
Q
what is emoblia cutis medicamentosa
A
vascular thrombosis from periarterial injection
3
Q
What drugs cause gingival hypertrophy
A
phenytoin, nifedipine, cyclosporine
4
Q
drugs that can cause telogen effluvium?
A
heparin, betablocker, IFN, lithium, retinoids
5
Q
drugs that cause anagen effluvium?
A
chemotherapy, heavy metals
6
Q
drugs that cause cutaneous lymphoid hyperplasia aka pseudolymphoma
A
anticonvulsants, neuroleptics, ARBS
7
Q
serum sickness eruption
A
antithymocyte globulin (prevention and treatment of acute rejection in organ transplantation and therapy of aplastic anemia)
8
Q
SDRIFE
A
betalactams, radiocontrast
9
Q
Radiation induced erythema multiforme or SJS
A
phenytoin and radiation
10
Q
first degree burn is
A
sunburn
11
Q
second degree burn is
A
divided into superfiical (non scarring) and deep (pale anesthetic skin) results in scarring
12
Q
striate palmoplantar keratoderma is AD or AR? What is the mutation (3)
A
desmoglein 1, desmoplakin, and keratin 1
13
Q
Oraloculargenital syndrome. Angular chelitis, seb derm, scrotal dermatitis. Deficiency in what?
A
Deficiency in B2 riboflavin
14
Q
Eruptive onset of multiple new nevi
A
Addison?s disease
15
Q
druginduced acanthosis nigricans
A
nicotinic acid, niacinamide, vitamin B3, somatotrophin, testosterone, oral contraceptives, insulin, protease inhibitors, and glucocorticoids
16
Q
Whats the malignancy associated with tripe palms? Tripe palms , acanthosis nigricans?
A
Lung cancer (tripe palms only), gastric cancer (tripe palms , Acanthosis nigricans)
17
Q
what diseases is anetoderma associated with?
A
50 percent will have antiphospholipid antibodies, lupus, Grave?s disease, scleroderma, hypocomplementemia, hypergammaglobulinemia, autoimmune hemolysis, and HIV
18
Q
secondary amyloidosis associated protein?
A
serum amyloid A
19
Q
Interferon alpha can in rare cases cause an exacerbation of what disease?
A
Psoriasis, spastic diplegia (in infants who were treating their hemangiomas)
20
Q
kwashiorkor is missing what? Marasmus is missing what?
A
Kwashiorkor is missing protein. Marasmus is missing more energy caloric intake
21
Q
you can get essential fatty acid deficiency if you have fat malabsorption, parental nutrition wo lipids and what other condition?
A
nephrotic syndrome
22
Q
how does essential fatty acid deficieny present?
A
like zinc and biotin deficiencies
23
Q
what fatty acids are deficient in essential fatty acid deficiency?
A
linoleic, linolenic, and arachidonic acid
24
Q
Vitamin A deficiency cutaneous findings?
A
phrynoderma (keratotis follicular papules resembing toadskin), blindness, bitot spots
25
Vitamin A excess findings?
SE with systemic retinoids alopecia, dermatitis, epistaxis, cheilitis
26
What systemic disease can cause betacarotene deposits in the skin?
Diabetes, nephrotic sndrome and hypothyroidism (impaired conversion of beta carotene to vitamin A)
27
Selenium deficiency causes what skin finding?
hypopigmentation of skin hair, leukonychia
28
vitamin B2 deficiency causes what skin findings?
oralgenital syndrome (cheilitis, angular stomatitis seb dermlike rash, tongue atrophy glossitis, genital and perinasal dermatitis)
29
Name vitamin B1, B2, B3, B6, B9.
thiamine 1, riboflavin 2, niacin 3, pyridoxine 6, folic acid 9
30
vitamin B3 deficiency causes what skin findings?
pellagra (casal s necklace, photosensitivity, 3D s (diarrhea, dementia, death))
31
What diseases and drugs causes vitamin B3 deficiency?
Hartnup, carcinoid, alcoholism, Azathioprine, Isoniazid, 5FU
32
vitamin B6 deficiency causes what skin findings?
Seb derm, angular chelitis
33
who s at highest risk for vitamin B6 deficiency?
alcoholics
34
who s at highest risk for folic acid deficiency? How does deficiency present?
goat milk diet. Deficiency presents as hyperpigmentation
35
Vitamin E excess can cause what?
petechiae and ecchymoses
36
Vitamin K deficiency can cause what?
purpura, ecchymoses, hemorrhage
37
Zinc deficiency SLC39A4 gene is inherited autosomal.
Autosomal recesive
38
Zinc deficiency has what lab value?
low alkaline phosphatase
39
Gouty tophi is an accumulation of what? most common found on the
Monosodium urate, Ear helix
40
Colloid milium stains like what?
Amyloid (congored, thioflavin t, crystal violet)
41
what is the best preservative for gout?
ethanol
42
milia like calcinosis on the dorsal hands face is associated with what disease?
down syndrome
43
Name 3 genetic syndromes that cause cutanous ulcers?
Adams oliver (aplasia cutis, CMTC), prolidase deficiency (leg ulcers) and klinefelter syndrome (leg ulcer)
44
Name 3 drugs that can cause cutaneous ulcers.
Hydroxyurea, interferon injection sites, MTX in psoraisis patients
45
Tx for cutanous small vesel vasculitis?
colchicine 0.6 3x day and dapsone 100mg day
46
Hypocomplementemic form of urticarial vasculitis has low serum levels of what complement? What antibodies do they have?
Low serum levels of C1q. They have antiC1Q antibodies
47
What two autoimmune diseases is Urticarial vasculitits associated with?
SLE and Sjogrens
48
Tx for urticarial vasculitis?
Indomethacin, dapsone, colchicine
49
Triggers of EED (infectious, autoimmune, hemologic)
betahemolytic strep, Hep B, HIV, TB, Syphillis, SLE, IBD, IgA paraporteinemia
50
pANCA stains for what? Describe the staining pattern.
Myeloperoxidase. Perinuclear staining
51
cANCA stains for what? Describe the staining pattern.
serine protease 3. granular cytoplasmic staining
52
which vasculitis has PGlike nodules and strawberry gums?
wegeners
53
which vasculitis is nongranulomatous
microscopic polyangiitis
54
which vasculitis has LCV , necrotizing granulomas with basophilic debris
wegeners granulomatosis
55
tx for wegener s granulomatosis?
cyclophosphamide (2mg kg day) , oral steroids (1mg kg per day)
56
which vasculitis is the most common cause of pulmonaryrenal syndrome? What is PRS?
Pumonary hemorrhage with focal segmental necrotizing glomerulonephritis
57
what are 2 possible drug triggers for churg strauss syndrome?
leukotriene inhibitors and omalizumab
58
which vasculitis has LCV with eosinophilic granulomas?
churgstrauss
59
What are the first symptoms of churg strauss?
adultonset asthma, nasal polyps, allergic rhinitis
60
whats the leading cause of death in churg stauss?
endocardiomyopathy
61
most common cause of death in PAN?
renal failure and HTN likely due to microaneurysm which are seen on angiography of mediumsized vessels
62
diagnosis critera for kawasaki?
5 days of fever, conjunctival injection, mucous membrane erythema, rash (perineal desquamation), cervical lymphadenopathy, peripheral edema erythema of hands feet
63
Tx for kawaskii? When do you have to give it to reduce coronary artery issues? Who doesn t respond well to treatment?
Within 10 days, highdose aspirin (100mg kg a day) , IVIG (2g kg) . Kids less than 1 year old do not respond well to treatment
64
What are the symptoms of temporal arteritis?
tenderness and erythema along sclap and temples with unilateral headache and loss of temporal pulse.
65
What are systemic findings associated with temporal arteritis?
Polymyalgia rheumatica with limb and muscle pain as well as vision loss
66
Describe the immunoglobulin for type 1, 2, 3 cryoglobulinemia
monoclonal IgM (1), monoclonal IgM polyclonal IgG, polyclonal IgG and polyclonal IgM
67
Describe the associations for type 1, 2, 3 cryoglobulinemia
lymphoproliferative disorders (type1), hepatitis C and autoimmune disease for type 2 and 3
68
Describe the histology for type 1, 2, 3 cryoglobulinemia
type 1 occlusive vasculopathy, type 2 3 LCV
69
Describe pathology for antiphospholipid syndrome
fibrin thrombi, lacks LCV
70
Whats the most sensitive ab for antiphospholipid syndrome
anticardiolipin
71
Whats the most specific ab for antiphospholipid syndrome
antiB2glycoprotein 1
72
cutanous collagenous vasculopathy pathology shows what? Stains with what?
thick hyalinized BMZ surrouding vessels stain positive with collagen 4 PASpositive
73
is cutaneous collagenous vasculopathy responsive to laser?
no it is not
74
what drug causes telangiectasias in sunexposied areas?
CCBs
75
erythromelalgia is associated with what possible lab value?
thrombocythemia
76
Cholesterol emboli presenting with what most commonly?
livedo reticularis or blue toe
77
Cholesterol emboli labs will show?
Eosinophilia, elevated BUN Cr
78
levamisoleinduced vasculitis will have what antibodies?
pANCA
79
degos disease presents in who? Clinical picture? Systemic symptomas?
young middleaged men as small ivory scars. Prediposed for GI bowel performation
80
Sneddon syndrome presents in who as what?
livedo racemosa with labile blood pressure and CNS disease (TIA, stroke, dementia). See in young WOMEN.
81
What is sneddon syndrome associated with? tx?
Antiphotopholipid syndrome. Tx with warfarin (INR 23)
82
Livedo racemosa associated with?
sneddon syndrom and apls
83
livedo reticularis associated with what drugs? What other syndromes?
amantadine
84
auriculotemporal nerve syndrome is the most common complication of what surgery?
parotidectomy
85
Drugs that cause erythema nodosum?
OCPs, sulfonamides, NSAIDs
86
Tx for IBDassociated EN?
TNF alpha inhibitors
87
Schmids Triad
pancreatic panniculitis, poly arthritis (intraosseous fat necrosis), eosinophilia
88
Tx for lipodermatosclerosis?
danazol, pentoxifylline
89
Cold panniculitis histology?
lobular panniculitis with pernio changes
90
type 1 5 alpha reductase is in
hair skin sebaceous glands
91
type 2 5 alpha reductase is in
prostate, inner root sheath of hair
92
shortened anagen phase and increase fibrous steamers? Name 3. Which of the 3 have the massive catagentelogen shift?
AGA, but AA, Trichotillomania has the most massive catagen shift
93
pemphigoid gestationis associated with what HLA?
HLA DR3, HLA DR4
94
Dermatosis of pregnancy that spares umbilicus?
Polymorphic eruption of pregnancy
95
Polymorphic eruption of pregnancy seen in what type of pregnancies?
1st pregnancy (primiparous women), multiple gestation pregnancies
96
tx for intrahepatic cholestasis of pregancy?
ursodeoxycholic acid
97
baby consequences for intrahepatic cholestasis of pregancy?
premature birth, intrapartum fetal distress, still birth
98
most common pruritc disorder of pregnancy?
atopic eruption of pregnancy
99
tx for trichotillomania?
Nacetylcysteine, clomipramine
100
What disease associated with AA is a prognostic of poor response?
atopic dermaittis
101
temportal triangular alopecia presents when? Look like?
Presents at birth or within the first decade as temporal sclap areas that lack terminal hairs
102
Congenital generalized lipodystrophy looks like what?
lipoatrophy in face, arms and legs with muscles
103
Familial partial lipodystrophy
lipohypertrophy on the face, lipoatrophy on the extremities
104
Acquired generalized lipodystrophy
congenital lipodystrophy , loss of fat on palms soles BUT, preserved bone marrow fat
105
Acquired partial lipodystrophy
exact opposite of familial partial. Gaunt face, lipohypertrophy lower extremities
106
All the lipodystrophies have an increase riask of diabetes insulin resistance. Which one has risk of hypertrophic cardiomyopathy?
congenital generalized
107
All the lipodystrophies have an increase riask of diabetes insulin resistance. Which one has fatal fatty liver and cirrhosis?
acquired generalized lipodystrophy
108
Which lipodystrophy has the 30% preceding Autoimmune connective tissue disease and panniculitis?
acquired generalized lipodystrophy
109
Which lipodystrophy is associated with membranoproliferative glomerulonephritis and has decrease in C3 complement?
Acquired partial lipodystrophy
110
which hairloss disorder has premature desquamation of inner root sheath, concentric lamellar fibroplasia and eccentric thinning of outer root sheath?
CCCA
111
LPP interface is at the level of the ?
infundibulum
112
LPP vs DLE histology?
interface dermatitis in DLE, but not in LPP
113
Structural hair abnormalies assocated with increased hair fragility?
bubble hair, moniletrix and the 4T s Trichorhexis invaginata, trichoexis nodosa, Trichothiodystrophy, PiliTorti
114
bubble hair due to
traumatic heat styling
115
monilethrix due to
Dsg 4 in AR inheritance. Hari cortex specific keratin in AD inheritance
116
moniletrix presents at what age?
normal hair at birth. Short brittle hair w perifollicular erythema and follicular hyperkeratosis a few months later
117
Pili tori is seen in what 4 diseases?
Cranberry, Blue, citrus, kinky noodles. Menkes kinky hair, Bazex Dupre, Crandall syndrome, Nethertons, urea cycle (cirullinemia, argininosuccinic aciduria)
118
Acquired pili torti seen in ?
anorexia nervosa and oral retinoids
119
Crandall syndrome
pili torti, hearing loss and hypogonadism
120
Natural history of pili torti?
normal hair at birth, replaced by brittle hair, then normalizes at puberty
121
BazexDupre Christol symptoms?
Pili torti, bCC, milia, follicular atrophoderma, hypohidrosis, hypotrichosis
122
Menkes Kinky hair?
pili torti, trichorrhexis nodosa, mental retardation, cupids bow upper lip, doughy skin, diffuse hypopigmentation (tyrosinase requires copper)
123
trichorrhexis invaginata seen in what disease?
nethertons SPINK5)serine protease inhibitor)
124
trichorrhexis nodosa seen in
congenital citrullinemia, argininosuccinic aciduria (both with pili torti, trichorrhexis nodosa and vomiting, seizures)
125
Trichothiodystrophy s hair features? Name 3
sulfur deficient, trichoschisis (transverse fractures) and alternating light dark under polarized light
126
Acquired progressive kinking of hair is a sign of?
AGA
127
Pili annulati
Light dark bands what you can see with naked eye (unlike trichothiodystrophy). Due to airfilled spaces
128
medicatoin induced hypertrichosis?
minoxidil, phenythoin and cyclosporine
129
hirsuitism on areola, lateral face and neck is likely due to what source?
ovarian
130
hirsuitism on abdomen and sterum is likely due to what source?
adrenal
131
PCOS labs
decreased FSH, increased LH, increased LH to FSH ratio greater than 3
132
onychorrhexis seen in
brittle nails. Seen in LP, chronic wet work, nailpolish use
133
onychauxis
subungal hyperkeratosis causing thickened nail in psoriasis, onychomycosis, eczema
134
onychocryptosis
ingrown nail
135
apparent leukonychia due to
nail bed edema
136
muehrcke s nails
chornic hypoaluminemia. Transverse white bands parallel to lunula
137
terry s nails
leukonychia o nail plate. Liver cirrhosis
138
half and half nails
leukonychia of half nail plate due to chronic renal disease
139
longitudinal melanonychia drugs and diseases? Name 4 drugs and 2 diseases
AZT, antimalarials, minocycline, gold, LaugierHunziker, Peutz jegher
140
longitudinal melanonychia caused by what onychomycosis bug?
Scytalidum
141
black nails
proteus mirabilis or trichophyton rubrum
142
green nails from what in the pseudomonas
pyocanin from pseudomonas
143
nail patella syndrome
traingle shaped lunula and absent patella. Need to see nephrology for possible nephropathy
144
yellow nail syndrome due to
lymphedema, pleural effusions, bronchiectasis
145
fordyce granules
ectopic sebaceous glands on the vermillion and buccal mucosa
146
geographic tongue
psoriasis
147
fissured tongue
melkerssonrosenthal (facial nerve paralysis, swelling of face, lips), down syndrome, cowden syndrome
148
necrotizing ulcerative gingivitis due to
malnutrition, immunosuppression, poor oral hygiene
149
number 1 tumor of the oral cavity?
fibroma along bite line
150
white sponge nevus due to mutation in
keratin 4 and 13
151
gingival enlargement due to
cyclosporine, phenytoin, CCB
152
orofacial granulomatosis
melkerssonrosenthal (facial nerve paralysis, fissured tongue), crohns, sarcoidosis
153
Cheiliis glandularis clinical picture?
enlargement of lower lip due to inflammation of secretory ducts
154
risk factor for cheiliis glandularis?
adult men with ho sun exposure, hence the increased risk of SCC
155
People with hypomelanosis of Ito have to worry about?
CNS, MSK or ophthalmologic abnormalities
156
third degree burn is
full thickness skin destroyed, forming an ulcer
157
fourth degree burn is
loss of skin and sucutaneous fat
158
Frost bite treatment
warm bath rapidly
159
Immediate pigment darking after sun exposure within the 1st 24hrs is due to
UVA. Photooxidation of melanin
160
Delayed tanning that develops over many days and lasts months is due to
UVB. Resulting in melanin synthesis
161
most common photosensitive dermatosis? due to what type of light
PMLE, due to UVA more than to UVB
162
most common cause of solar urticaria? What type of light?
visible light
163
Porphyria cutanea tarda due to decrease in what enzyme?
uroporphyrinogen decarboxylase (UROD)
164
PCT plasma fluoresces at what nm?
620nm
165
Triggers for PCT (5)
Hemochromatosis ,Hepatitis C, alcohol abuse, estrogen, HIV
166
homozygous mutation of UROD is what disease?
hepatoerythropoietic porphyria (HEP)
167
congenital erythropoietic porphyria due to what mutation?
UROS (uroporphyrinogen 3 synthase). Gunther s disease
168
Porphyria with red urine? What fluorescence nm
congenital erythropoietic porphyria. Excited by visible light at 410
169
Tx for congenital erythropoietic porphyria?
Iron chelation w deferoxamine, ascorbic acid, alphatocopherol
170
most common porphyria in children?
erythropoietic protoporphyria (EPP)
171
EPP due to mutatoin in what?
ferrochelatase in the mitochondria
172
Besides the skin, what other problems do kids with EPP have?
Cholelithiasis (protoporphyrins accumulate in the liver causing hepatotoxicity and progressive hepatic dysfunction)
173
EPP treatment?
photoprotection and oral Betacarotene, plasmapheresis, liver transplantation
174
Cauliflower ear
due to recurrent trauma to external ear. Often in wrestlers
175
amyloid stains for what 4
congo red, crystal violet, thioflavin T, PAS
176
Percent of nodular amyloid that can progress to systemic amyloid?
7 percent
177
Tx for biliary pruritus
cholestyramine, ursodiol
178
Aquagenic puritus presents as? Tx?
itch and pain after contacting water. No visible skin changes. Treat with clonidine and propranolol. (both HTN meds)
179
Meralgia paresthetica due compression of what nerve?
Lateral femoral cutaneous nerve as it passes under the inguinal ligament
180
Trigeminal neuralgia due to injury to what?
gasserian ganglion that can happen after treatment for trigeminal neuralgia
181
Familial dysautonomia syndrome symptoms
autonomic and sensory dysfunction. Cant produce tears, cant taste food, cant salivate, cant feel pain.
182
Aquagenic PPK presents as?
Thick clearwhite pebblelike palmar eruption afte rwater immersion
183
Aquagenic PPK associated with?
sun burn
184
Circumscribed palmar plantar hypokeratosis presents as? What do we see on histopath?
Welldefined pink circular depresson on palm or sole. On histopath, appears as focally decreased statum corneum.
185
malignant polyps in stomach or small intestine
Peutz Jegher
186
recurrent epistaxis
Osler weber rendu
187
another name for tyrosinemia type 2
(Richner Hanhart syndrome)
188
symptoms of richner handhurt
ocular findings of keratitis with photophobia, corneal ulceration. Cutaneous manifestations include painful palmoplantar keratosis
189
Genital lentigines and mucosal lentigines and longitudinal melanonychia
LaugierHunziker syndrome
190
Lentigines syndrome with endocrinopathies
CARNEY LAMB. endocrinopathies (hyperactive endocrine activity due to tumors of the adrenals, pituitary, and other endocrine gland)
191
Muir Torre syndrome is associated with what two cancers?
colon and genitourinary
192
what is the first sign of neuro invovlement in XP?
decreased or absent deep tendon reflexes and hearing loss at higher frequencies
193
pachydermoperiostosis
pachydermia, periostosis, and finger clubbing. Onset is often at puberty
194
CHILD syndrome, ConradiHunermann syndrome, Goltz syndrome, and incontinentia pigmenti are all inherited
Xlinked Dominant
195
