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Clinical Dermatology > Gen Derm and Pediatrics 2 > Flashcards

Gen Derm and Pediatrics 2 Flashcards

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1
Q

medication that causes inflamed seb keratoses

A

cytarabine and taxanes

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2
Q

how is serum-like sickness different form serum sickness?

A

real serum sickness has hypocomplementemia, circulating immune complexes, vasculitis and renal dysfunction

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3
Q

above or below periosteum? Caput succeduaneum and cephalohematoma?

A

Caput is above the periosteum and cephalohematoma is below the periosteum

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4
Q

Subgaleal hematoma is dangerous because it can

A

lead to DIC, shock, anemia. Need inpatient monitoring

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5
Q

Name the 3 stages of transient neonatal pustular melanosis

A

Pusutules w/o erythema, then collarettes of scale, then hyperpigmented macules

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6
Q

Transient neonatal melanosis and etox. Which one is at birth?

A

Transient melanosis at birth. Etox usually at 24 hrs.

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7
Q

Epstein vs bohn. Which on is on the palatal raphe, and which is on the alveolar ridge?

A

Epistein is a pearl on the palatal raphe. Bohn is along the gum margin

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8
Q

Collagenoma can be seen in what genodermatoses?

A

Shagreen path in tuberous sclerosis and cerebriform collagenoma in Proteus syndrome.

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9
Q

Familial cutaneous collagenoma syndrome (3)

A

collagenoma, hypogonadism and cardiomyopathy

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10
Q

Elastin connective tissue nevus can be seen in what genodermatoses?

A

Buschke-Ollendorff

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11
Q

Becker’s nevus is associated with what of the underlying structures?

A

breast hypoplasia

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12
Q

aplasia cutis is associated with what drug? What infection?what 2 genoderms?

A

Methimazole, HSV/VZV, adams oliver , bart syndrome

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13
Q

describe adams oliver syndrome

A

aplasia cutis w/ cranial defect, CMTC, congenital heart defect, limb abnormalities (syndactyl, brachdactyl, oligodactyl)

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14
Q

bart syndrome

A

aplasia cutis with dominant dystrophic epidermolysis bullosa

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15
Q

Nasal glioma is

A

ectopic neuroectoderm

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16
Q

Difference between meningocele and encephalocele?

A

Encephalocele includes brain (glial tissue).

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17
Q

Which one will transluminate: nasal glioma, meningocele, dermoid cyst?

A

meningocele

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18
Q

Accessory tragus is d/t faulty development of

A

the first brachial arch

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19
Q

Goldenhar syndrome is due to abnormal development of

A

the first and second brachial arch

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20
Q

goldenhar syndrome (3)

A

abnormal eyes, ears, and spine. Facial asymmetry, hearing impairment, hypoplasia of the maxilla

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21
Q

where are congenital rest of the neck?

A

along the anterior border of the sternocleiomastoid

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22
Q

Congential rubella is most severe when the mom is infected durign what period

A

first 12 weeks of gestation

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23
Q

Congenital toxoplasmossis has what 3 head and neck findings?

A

Chorrioretinitis, hydrocephalus, intracranial calcification

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24
Q

Treatment for congenital toxo?

A

Pyrimrthamin, sulfadiazine, folinic acid for 1 year

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25
Q

What is the most common congenital infection and cause of extramedullary hematopoeisis?

A

Congenital CMV, IV ganciclovir

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26
Q

Is this early or late congenital syphilis? (2yo): erosions on hands/feet and periorifical

A

early syphilis

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27
Q

Is this early or late congenital syphilis? (2yo): secondary syphilis like papulosuqamous lesions

A

early syphilis

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28
Q

Is this early or late congenital syphilis? (2yo): snuffles. What is snuffles?

A

early syphilis. Snuffles if persistent rhinitis with blood and white discharge

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29
Q

Is this early or late congenital syphilis? (2yo): mucous patches

A

early syphilic

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30
Q

Is this early or late congenital syphilis? (2yo): parrot pseduoparalysis. What is that?

A

early syphilic. Decreased movemenet of the upper limbs d/t pain. Arms are at sides.

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31
Q

Is this early or late congenital syphilis? (2yo): saber shins

A

late syphilis

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32
Q

Is this early or late congenital syphilis? (2yo): higoumenakis sign

A

late syphilis. Enlargement of medial clavicle

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33
Q

Is this early or late congenital syphilis? (2yo): mulberry molars, hutchinson notched teeth

A

late syphilis.

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34
Q

Is this early or late congenital syphilis? (2yo): saddle nose

A

late syphilis.

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35
Q

Is this early or late congenital syphilis? (2yo): perioral rhagades

A

late syphilis.

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36
Q

Is this early or late congenital syphilis? (2yo): clutton joins

A

late syphilis. Painless, symmetric swelling of knees

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37
Q

Congenital varicella is separated into fetal and neonatal infection. How is this defined?

A

Fetal VZV is within 20 weeks of gestation (like 20 dermatomes). Neonatal VZV is 7 days before, and 2 days after delivery.

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38
Q

Fetal varicella presents as? Prognosis?

A

Presents at birth with deep stellate scars, limb paresis, catarcts, micropthalmia, hydrocephalus. Prognosis depends.

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39
Q

Neonatal varicella presents as? Prognosis?

A

Presents within 2 weeks with vesicles, generalized. Fatality of 30% if infection is <5 days. After 5 days has a benign course.

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40
Q

Congenital herpes is separated into fetal and neonatal infection. How is this defined?

A

Fetal is d/t ascending infection. Neonatal is d/t infection during brith or perinatal period

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41
Q

Congenital herpes prognosis? Feal and neonatal

A

Fetal has 75% mortality if untreated. Neonatal herpes has 50% CNS infection and neurologic sequelae

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42
Q

Rubeloa virus? RNA, DNA?

A

Measeles. Paramyovirus ssRNA

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43
Q

Mealses incubation period?

A

1-2 weeks, then cough/coryza/conjunctivitis

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44
Q

Possible complication of measles?

A

encephaliits, myocardidits, and subacute sclerosing panencephalitis

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45
Q

Measles vaccine schedule

A

first at 1 year old, second at 5 years old

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46
Q

Rubella mucocutaneous findings include?

A

Forchheimer’s spots (hard palate petechiae) and painful lymphadenopathy

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47
Q

Possibe complications of German measles?

A

Arthritis and arthralgias most common.

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48
Q

other names for slapped cheek diesease?

A

5th disease, erythema infectiosum, slapped cheek

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49
Q

slapped cheek caused by what virus?

A

parvo b19, ssDNA

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50
Q

what viral exanthem is not contageious when it skin eruption develops?

A

5th disease, erythema infectiosum, slapped cheek. Skin eruption coincides w/ development of IgG

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51
Q

Adults with slapped cheek, parvo b19 will develop what?

A

arthritis with small joint predominance

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52
Q

patients with petechiae and purpural that is sharply demarcated at the wrist. Can they go to school?

A

No, ParvoB19 virus is viremic at the time of skin eruption.

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53
Q

other names for 6th disease?

A

6th disease, roseaola infantum, exanthem subitum.

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54
Q

roseola d/t what virus?

A

HHV6.

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55
Q

roseola clinical picture?

A

incubate for 1-2 weeks, then high fever for 5 days, causing febrile seizures, nagayama spots

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56
Q

what are nagayama spots?

A

red macules on soft palate and uvula

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57
Q

Possible sequelae of roseola?

A

DRESS, because the HHV 6 remains latent in CD4 T cells

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58
Q

Coxsackie virus is part of what family of viruses?

A

PICORNA, along with polio, enterovirus, echovirus and hepatitis A

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59
Q

traditional Hand foot mouth caused by what coxsakie virus? Atypical one?

A

Traditional one caused by coxsakie a 16, EV17. Now the atypical ones are caused by coxsakie A6

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60
Q

Chronic mucocutanoeus candidiasis can be seen in APECED syndrome.

A

Autimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome

61
Q

APECED syndrome d/t mutation of what?

A

AIRE (AutoImmuneRegulator) mutation which results in failture of T cell tolerance with resultant autoimmunity

62
Q

APECED syndrome features include?

A

Hypoparathyroidism, alopecia areata, vitiligo, pernicious anemia

63
Q

OCA1a and 1b d/t mutations in what?

A

TYR (absent in type 1a, decreased to 10% of normal level in type 1b)

64
Q

Temperature senstive OCA is what type?

A

OCA1b subtype (obvi, cuz they have some pigment)

65
Q

What OCA type is the most common, whats the mutation?

A

OCA type 2, seen in africans. (P gene).

66
Q

Prader Willi and Angelman syndrome + 2nd mutation give you

A

OCA 2 like hypopigmentation in 1% of PW and angelman patients

67
Q

OCA, Chediak Higashi, Griscelli, Hermansky Pudlak are all inherited how?

A

Autosomal recessive

68
Q

Griscelli 1,2,3 associated with what? Least severe Griscelli

A

GS1 severe neurologic impairment, GS2 (combined immunodeficiency), GS3 (cutaneous- least severe)

69
Q

Most common type of OCA in japan?

A

OCA type 4 due to mutation in SLC45A2

70
Q

Chediak Higashi Syndrome presents with what syndrome?

A

Silverly hair, OCA, immunodeficiency, bleeding diathesis, neurologic degeneration

71
Q

Chediak Higashi patients have a poor prognosis. When do they die, from what?

A

Die at age 10 as a result of lymphoproliferative accelerated phase/hemophagocytic syndrome

72
Q

Whats Elejalde syndrome?

A

Variant of Gricelli Syndrome 1 with severe neurologic dysfunction, but no immunodeficiency

73
Q

Hermansky Pudlak syndrome composed of what symptoms?

A

Nosebleeds, menorrhagia, granulomatous colitis, progressive pulm fibrosis, renal failture d/t lysosomal ceroid lipofuscin

74
Q

Griscelli syndrome d/t what global cellular defect? What does trichoscopy show?

A

D/t aberrant translocation of melanosome along microtubules causing larger, irregularly clumped melanin

75
Q

Chediak Higashi d/t what global cellular defect? What does trichoscopy show?

A

D/t impaired biosynthesis and storage of melanosomes, platet granules. Trichoscopy shows regularly spaced melanin

76
Q

Melanocytes with giant melanosome seen in?

A

Chediak Higashi

77
Q

Which griscelli has a accelerated phase /HLH?

A

GS2

78
Q

Most common cause of death in Hermansky Pudlak?

A

pulmonary fibrosis

79
Q

Which pigmentary disorder shows an absense of dense bodies in platelets on EM?

A

Hermaky Pudlak

80
Q

Waardenburg is inherited AR or AD?

A

Inherited AD

81
Q

Waardenburg syndrome 1. Mutation and what clinical symptoms?

A

PAX3. White forelock, unibrow, different iris colors, dystopia canthorum, deafness

82
Q

Waardenburg syndrome 2. Mutation and what clinical symptoms?

A

MITF. Like WS1 minus dystopia canthorum. More likely to be deaf.

83
Q

Waardenburg syndrome 3. Mutation and what clinical symptoms?

A

PAX3. WS1 with hypoplasia, contracture and syndactyly of the fingers.

84
Q

Waardenburg syndrome 4. Mutation and what clinical symptoms?

A

SOX10. WS1 plus hirschsprung’s disease

85
Q

McCune Albright is inherited how? Mutation in what gene?

A

Trick. It is not inherited. It is a post zygotic mutation in GNAS1

86
Q

Describe polyostotic fibrous dysplasia of McCune albright.

A

Usually under the CALM. Manifests as pain, deformity, recurrent fractures.

87
Q

Endocrine abnormalities of McCune Albright include what?

A

precocious puberty, hypophosphatemic rickets, infantile Cushing.

88
Q

Dowling Degos, inherited how? Mutation in what?

A

Inherited AD. Mutation in Keratin 5

89
Q

How does dowling degos present? What’s on the neck/back?

A

Presents during adulthood w/ reticulated hyperpigmentation involving axilla and groin. With comedones on the neck/back

90
Q

Mutation in dyschormatosis symmetrica hereditaria?

A

ADAR gene. Encodes RNA specific adenosine deaminase. Dyspigmentation on extremities and face

91
Q

Dyskeratosis congenita inherited how?

A

XLR (most common)

92
Q

Dyskeratosis congenita mnmonic

A

TeLOMPuPaPi (telomere, leukoplakia, onychodystrophy, boneMarrow failure, Pulmonary fibrosis, Pigmentation, Palmoplantar hyperkeratosis)

93
Q

Dyskeratosis congenita has increased risk of what malignancies?

A

Hematologic malignancy, squamous cell of oral mucosa

94
Q

Dyskeratosis congenita pt die on average at 16yo. What are causes?

A

Pancytopenia d/t BM failure, SCC/hematologic malignancy, pulmonary fibrosis

95
Q

Naegeli-Franceschetii-Jadassohn Syndrome d/t mutation in? Clinical?

A

Mutation in Keratin 14 (like EBS w/ mottle). Hyperpigmentation while a child, dental anomalies (loss of teeth), PPK

96
Q

Dermatopathica pigmentosa reticularis d/t mutaiton in? Clinical?

A

Mutation in Keratin 14 (like EBS w/ mottle). Hyperpigmentation permanent and diffuse non-scarring alopecia

97
Q

All the lentigiene syndrome are inherited how?

A

Autosomal dominant

98
Q

multiple granular cell tumors associated with

A

LEOPARD syndrome

99
Q

LEOPARD syndrome mnemonic.

A

lentigienes, EKG (cardiomyopathy), ocular hypertelorism, pulmonic stenosis, abnormal genitalia (hypogonad, cryptorchidism), retardation of growth, deafness

100
Q

Primary pigmented adrenocoritcal disease, Sertolic cell tumor, Psammomatous schwannoma seen in what syndrome?

A

Carney complex

101
Q

What are the GI symptoms with Peutz Jegher?

A

GI polyps in jejunuma nd ileum, causing intussusception

102
Q

What other cancers in Peutz Jegher?

A

GI, lung, breast.

103
Q

Cronkhite-Canada symptoms?

A

Hair Skin nails (alopecia, lentigines, nail dystrophy) + intestinal polyposis

104
Q

Banana Riley mutatoin and clinical symptoms?

A

PTEN, penile lentignese, lipomas, hydrocephaly, also with intestinal polyps

105
Q

All the tumor syndromes (Gorlin, Birt Hogg, Cowden, Gardner, Brook) are inherited how?

A

Autosomal dominant

106
Q

Gorlin is a mutation in what gene that encodes for what?

A

PTCH gene that encodes for the PATCH protein which suppresses SHH

107
Q

What’s the major criteria for Gorlin syndrome?

A

More than 2 BCC (or 1 before 20yo), plamoplantar pits, odontogenic keratocysts, calcification of falx, medulloblastoma, 1st degree relative

108
Q

minor criteria for Gorlin syndrome includes?

A

Frontal boss, bifid ribs, ovaria/caridac fibroma

109
Q

Name 5 syndromes w/ multiple BCC

A

Gorlins, Bazex Dupre, Rombo, Brooke Spiegler, Scheopf Schulz Passarge

110
Q

Birt Hogg Dube Associations

A

FAT hogg RoLs: Fibrofolliculoma, Acrochordons, Trichodiscomas, RCC, spontaneous recurrent pneumothorax

111
Q

Brooke Spiegler Associations

A

Red, White Blue Spiralizer (Cylindroma, Trichoep, BCC, Spiroadenoma) Salivary and parotid gland tumor.

112
Q

MEN 1 has cutanous findings similar to?

A

Tuberous sclerosis + Pit Par Pan

113
Q

Men 2a and 2b both have? What else do they have?

A

Pheochromocytoma, and medullary thyroid carcinoma. 2a has parathyroid hyperplasia, 2b has mucosa neuromas

114
Q

What cutanoue findings do Men 2a have?

A

lichen and macular amyloidosis

115
Q

name 5 cutaneous findings for cowdens

A

Sclerotic fibroma, lipoma, palmoplantar keratoese, acral keratoses, tricholemmomas,

116
Q

Most common malignancy in cowdens, other malig

A

breast adenocarcinoma, follicular thyroid carcinoma, endometrial carcinoma

117
Q

Lhermitte Duclos Disease is pathognomoic for?

A

Cowdens. Tumor of the cerebellum. Overgrowth of cerebellar ganglioin cells

118
Q

Gardner syndrome cutaneous manifestations include?

A

epidermoid cysts, pilomatricoma, lipomas, painless osteomas of the skull/mandible

119
Q

What non-cutaneous findings is Gardner syndrome associated with?

A

GI adenocarcinoma (prophylactic colectomy needed), desmoid tumor blocks ureter or Small bowel, CHRPE (congenital hypertrophy of retinal pigment epithelium)

120
Q

EB SImplex inheritance?

A

AD except for EBS with muscular dystrophy AR

121
Q

Junctional EB inheritance?

A

AR

122
Q

Dystrophic EB inheritance?

A

AR except for DDEB

123
Q

EBS mutatoin?

A

keratin 5/14 except for EBS w/ muscular dystrophy which is plectin

124
Q

EBS localized and generalized symptoms?

A

tense bullae on hands, feet, friction. No scarring. Some oral bulle in the generalized

125
Q

EBS generalized severe symptoms?

A

Clustered hyerpetiform bullae. Some scarring. Oral blisters, nail dystrophy, palmoplantar hyperkeratosis, clumped tonofilaments on EM

126
Q

When does EBS with muscular dystrophy present?

A

muscle weakness is delayed (develop in infancy through adulthood)

127
Q

Rarest form of EB?

A

JEB

128
Q

What do you find in all forms of JEB?

A

enamel hypoplasia/pitting

129
Q

What are the mutations in JEB? Generalized severe, Gen-inter, pyloric atresia

A

Generalized severe (laminin 332), Generalized intermediate (Laminin 332 + BPAG2), JEB pyloric atresia (a6b4 integrin)

130
Q

JEB generalized severe cutaneous and non cutaneous features? Prognosis

A

Perioral blisters (which becomes perioral grnaulation tissue), hoarse cry, death within first few years from respiratory failure or sepsis.

131
Q

JEB generalized intermediate cutaneous and non cutaneous features? Prognosis

A

Generalized blisters on skin and mouth, which heal with atrophic scars. Also has scarring alopecia. Survive to adulthood

132
Q

JEB w pyloric atresia associated clinicla features?

A

Pyloric atresia, Ureteral stenosis (scarred urinary tract)

133
Q

Dystrophic EB mutation?

A

Collagen 7 (complete lack of anchoring fibrils in RDEB, generalized)

134
Q

Dominant DEB features?

A

Atophic scarring, milia, NAIL DYSTROPHY

135
Q

Recessvie DEB features? Die from?

A

Mitten deformities, esophageal strictures, renal failure. Die from SCC, then it’s Renal failure

136
Q

Kindler Syndrome Features?

A

Blisters and photosensitivty and poikiloderma.

137
Q

What do you see in Kindler syndrome on electron microscopy?

A

duplication of lamina densa. FERMT gene

138
Q

PHACES stands for

A

Posterior fossa malformation, hemangioma, arterial anomalies, coarctation of aora, eye (cataracts), sternal cleft

139
Q

Suspect neonatal hemangiomatosis if more than how many hemangiomas

A

If 5 or more hemangiomas

140
Q

Neonatal hemagiomatosis can lead to

A

Liver failture or high output congestive heart failture

141
Q

Tx for Kasabach Merritt Phenomenon?

A

Vincristine and Systemic steroids

142
Q

Struge Weber mutatoin

A

somatic mosaic mutation in GNAQ

143
Q

Head CT shows what in Sturge Webeer?

A

Tram Tracking

144
Q

Most common form of Phakomatosis Pigmento vascularis? What is it associated with?

A

Type 2. Associated with Sturge Weber, Parkes Weber or KTS

145
Q

Phakomatosis pigmentokeratotica

A

Nevus spilus in conjuection with nevus sebaceous. Hypophosphatemic vitamin D resistant rickets

146
Q

Klippel Tresaunay Syndrome complications include

A

High output cardiac failure

147
Q

What tumors are proteus syndrome ppl at risk for?

A

Bilateral ovarian cystadenoma

148
Q

What cutaneous findings do we see in Beckwith Wiedemann?

A

Facial capillary malformation, macroglossia, anterior earlobe crease

149
Q

<p>What tumors are Beckwith Wiedmann kids at risk for?</p>

A

Wilms tumor, rhabdomyosarcoma, neuroblastoma

Clinical Dermatology (11 decks)

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