General Dermatology Chapter 3 Flashcards
1
Q
List the 6 hair disorders with + fragility?
A
Bubble hair
Monilethrix, “beaded hair”
Pili torti, “tortuous hair”
“Bamboo hair”
Trichorrhexis nodosa, “broomstick hair”
Trichothiodystrophy, “tiger tail hair”
2
Q
Most common mutation identified in LCH lesions?
A
BRAF V600E
3
Q
Factor XIIIa is found in?
A
NON-LCH conditions:
Juvenile xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, and Erdheim-Chester disease
4
Q
LCH tx?
A
BRAF V600e mutation not present, cutaneous LCH can be treated with immunosuppressing agents like corticosteroids, thalidomide, azathioprine, and MTX rather than vemurafenib, a BRAF kinase inhibitor that may be a valuable treatment for LCH patients with severe multisystem disease and detected BRAF V600e mutation.
5
Q
A
Traumatic anserine folliculosis (TAF). Tx: Tretinoin 0.025%
6
Q
PTCH1 mutations have been found in?
A
Gorlin syndrome, sporadic trichoepitheliomas, bladder carcinoma, and SCC of the esophagus
7
Q
Accordion hand is a radiologic finding characteristic of?
A
Multicentric reticulohistiocytosis
8
Q
Porphyria cutanea tarda can be a marker for?
A
Hereditary hemochromatosis
9
Q
Costello syndrome have an increased risk of developing which?
A. SCC
B. Renal cell carcinoma
C. Rhabdomyosarcoma
D. Pneumothorax
E. Cardiac myxomas
A
C. Rhabdomyosarcoma
10
Q
Which of these dermatophytes causes Tinea capitis without hair loss?
A. Trichphyton violacium
B. Microsporum lanosum
C. Epidermophyton floccusum
D. Trichophyton gypsum
E. Trichophyton rubrum
A
A. Trichphyton violacium
11
Q
spindle lipoma’s develop on the?
A
posterior neck, upper back and shoulders
12
Q
Which of the following is MOST likely to be found on physical examination of a patient with Gronblad – strandberg syndrome ?
A. Splenomegaly
B. Angioid streaks
C. Lymphadenopathy
D. Palpable purpura
E. Scaly plaques on the knees
A
B. Angioid streaks
13
Q
Which of the following is associated with seborrhea?
A. Scleroderma
B. Pachydermoperiostosis
C. Steatocytoma multiplex
D. Piezogenic pedal papules E. Pseudoxanthoma elasticum
A
B. Pachydermoperiostosis
14
Q
Maculae caeruleae (blue dots on the thigh) occur in Which of the following?
A. Tungiasis
B. Larva migrans
C. Onchocericoma
D. Pediculosis pubis
E. Cutaneous schistosomiasis
A
D. Pediculosis pubis
15
Q
Waldenstrom hypergamma globulinemic purpura is most frequently associated with?
A. Rheumatoid arthritis
B. Lupus erythematosus
C. Lymphoma
D. Multiple myeloma
E. Sjogren’s syndrome
A
E. Sjogren’s syndrome
16
Q
What disorders are associated with IgA gammopathy?
A
Sweet’s syndrome
Pyoderma gangrenosum
IgA pemphigus and subcorneal
pustular dermatosis
Erythema elevatum diutinum
POEMS syndrome
17
Q
What disorders are associated with IgM gammopathy?
A
Schnitzler’s syndrome
Waldenström macroglobulinemia
Cryoglobulinemia
18
Q
IgG-k is associated with?
A
Necrobiotic xanthogranuloma
Scleredema
19
Q
IgG-lamda is associated with?
A
Scleromyxedema
20
Q
Secondary eruptive xanthomas due to what drugs?
A
Oral retinoids, protease inhibitors, olanzapine, estrogen replacement
21
Q
Tuberous xanthomas mainly occur on?
A
Elbows and knees; a/w type II and III
22
Q
Firm nodules on Achilles tendon are?
A
Tendinous xanthomas seen in type II hyperlipidemia
23
Q
Dysbetalipoproteinemia assoc. with Tuberoeruptive, tuberous, plane, palmar creases?
A
Type III
24
Q
Histology of xanthomas?
A
Foam cells, macrophages w/ lipidized cytoplasm, in dermis
25
Preformed mediators?
"Preformed HHP"
Heparin
Histamine
Proteases
26
Urticaria include immune complex deposition, urticarial vasculitis, and what other meds?
NSAIDs, radiocontrast media, polymyxin B, ACE inhibitors (due to ↑ bradykinin), and dietary pseudoallergens
27
Most common drug causing urticaria?
b-lactams
28
Blanched vasoconstricted halos
around pink wheal
○ Responds better to propranolol than to antihistamines
○ Can reproduce lesions by intradermal
norepinephrine injections
29
Aquagenic urticaria can be commonly seen with?
cystic fibrosis
30
Schnitzler’s syndrome?
Chronic urticaria (burn > itch), fevers, bone pain, arthralgia/arthritis, ↑ ESR, and IgM gammopathy
■ Neutrophilic infiltrate on histopathology; anakinra is a good treatment part of urticarial vasculitis
31
Hereditary angioedema without urticaria?
■ Types I and II due to mutations in C1 inh
- Type I has ↓ C1 inh levels
- Type II has ↓ C1 inh function
■ Type III due to an activating mutation in Hageman factor (FXII)
■ All forms of HAE are autosomal dominant
32
↓ C1q levels?
seen in AAE only!
↓ C1 inh in type I HAE*
33
C1 inh deficiency tx?
Oral danazol is ToC for prophylaxis and C1 inh concentrate is ToC for acute attacks
34
Five major subtypes of Sweet's syndrome?
Classic (60%–70%), cancer-associated
(10%–20%), inflammatory disease-related (10%–15%), drug-induced (5%), and pregnancy (2%)
35
For classic sweet's gender and location predilection?
F > M, 3:1
Favors head, neck and upper extremities
36
Extracutaneous features of sweet's?
Fever (50%–80%), malaise,
preceding URI or flu-like symptoms, leukocytosis (70%), arthralgias/arthritis, ocular involvement (conjunctivitis, episcleritis, scleritis, retinal vasculitis, and iridioyclitis), cardiac involvement (aortitis, cardiomegaly, coronary artery occlusion)
37
Lab abnormalities in Sweet's?
■ ↑ ESR/CRP (90%)
■ Leukocytosis: neutrophilia
w/ ↑ band forms “left shift”
38
Triggers for sweet's?
Infections: streptococcus, yersiniosis; HIV, hepatitis C
Drugs: G-CSF, GM-CSF, ATRA, TMP-SMX, minocycline, OCPs
Cancer: AML
39
Histiocytoid Sweet’s will show infiltrate of?
Deep dermal infiltrate of CD163+ macrophages
40
Mimickers of Sweet’s syndrome?
* CANDLE syndrome (Chronic Atypical Neutrophilic Dermatosis with Lipoatrophy and Elevated temperature): autoinflammatory disorder of children with mutations in PSMB8 gene that can mimic Sweet’s
* Marshall syndrome: rare childhood disease that has Sweet’s-like lesions that resolve w/ acquired cutis laxa at affected sites
41
Pyoderma gangrenosum in kids?
on head and anogenital region
a/w IBD or leukaemia
42
Bullous PG?
More superficial, less destructive than classic PG, More widespread, a/w hematologic malignancy (AML,
CML, MDS, polycythemia vera)
43
Painless cribriform ulcers w/
verrucous growths on trunk of PG?
Vegetative PG
44
What's the cause of Behçet's disease?
Multifactorial (infectious trigger?); circulating immune complexes and neutrophil dysregulation → vascular injury 1st common sx: aphthous stomatitis
45
Behcet's number cause of mortality?
Ocular (90%), including blindness
46
MAGIC syndrome?
MAGIC syndrome = features of Behçet’s + relapsing polychondritis
47
Hypereosinophilic syndrome (HES) criteria?
■ Peripheral blood eosinophilia, eosinophils >1500/mm3 and/or tissue hypereosinophilia (e.g., eosinophils
> 20% of cells in bone marrow)
■ No evidence of infectious, allergic, or other underlying causes
■ Symptoms/signs of end-organ involvement (due to eosinophil products)
HES divided into primary (neoplastic; 75%) versus secondary (reactive; 25%)
48
Neoplastic Hypereosinophilic syndrome fusion gene?
FIP1L1-PDGFRA fusion gene, tx with Imatinib if fusion gene present
49
Hypereosinophilic syndrome die from?
Congestive heart failure, they need regular echocardiograms
50
Immune reaction to antigen (e.g., tinea pedis, other dermatophyte infections, other fungi, viruses, parasites), drug, pregnancy, malignancies?
Erythema annulare centrifugum
51
Mild spongiosis, focal parakeratosis, and perivascular lymphohistiocytic infiltration, which is tight and dense "coat sleeve” is seen in?
Erythema annulare centrifugum
52
Agar for Borrelia?
Barbour-Stoenner-Kelly medium
53
Most common malignancy in Erythema gyratum repens?
Lung (most common) > breast and GI
Lesions develop 1 year pre-cancer to 1 year post-cancer diagnosis, and resolve once cancer treated
54
Cutibacterium acnes activates TLR-2 on macrophages then activates what part of the inflammasome?
NLRP3 of neutrophils
55
Effect of tretrinoin on acne?
downregulate TLR-2 expression
56
Severe eruptive nodulocystic acne, without systemic manifestations (vs. acne fulminans)?
Acne conglobata
57
Common sites for neonatal acne?
Cheeks and nasal bridge
58
Infantile acne tx?
Topical retinoids, topical antibiotics, or in moderate-severe cases azithromycin or isotretinoin
59
Type of occupational acne caused by exposure to chlorinated aromatic hydrocarbons (found in electrical conductors, insulators, and insecticides/fungicides/ herbicides)?
Chloracne, preferred sites are face, neck (including retroauricular),
axilla, scrotum, and penis
60
SAPHO (Chronic recurrent multifocal osteomyelitis) stands for?
Synovitis
Acne
Pustulosis
Hyperostosis
Osteitis
61
PAPA syndrome AD mutation in?
CD2-binding protein 1 (CD2BP1; aka PSTPIP1)
62
“bull’s head” sign may be seen on X-ray?
SAPHO (Chronic recurrent multifocal osteomyelitis)
63
HAIR-AN?
Hyper Androgenism
Insulin Resistance
Acanthosis Nigricans
Unique subtype of PCOS
64
Mutation in fibroblast growth factor receptor 2, FGFR2?
Apert syndrome (Acrocephalosyndactyly)
65
Molecules inflamed in rosacea?
Kallikrein 5, TPRV, cathelicidin LL-37, and TLR2 are upregulated
66
Haber’s syndrome?
Genodermatosis w/ rosacea-like eruption and verrucous lesions
67
common in Asians, Smooth, firm, yellow-brown to red 1 to 3 mm
monomorphous papules with apple jelly color on diascopy in butterfly distribution, heals w/scarring is likely?
Lupus miliaris disseminatus faciei
68
Gram-negative folliculitis is seen in?
Patients receiving prolonged antibiotic treatment (esp. tetracyclines)
69
Eosinophilic pustular folliculitis (Ofuji’s disease) + Peripheral eosinophilia; Tx: oral indomethacin
70
Tx of Trichodysplasia spinulosa?
Topical cidofivir, tazarotene gel, oral
valganciclovir
71
Tx of Pseudofolliculitis barbae?
Bunch of tiny curled hairs
Tx: Stop shaving; laser hair removal,
72
Follicular occlusion tetrad?
Acne conglobata
Hidradenitis suppurativa
Dissecting cellulitis of the scalp
Pilonidal cyst
73
HS mutation?
"Gamma-secretase complex" (nicastrin, presenilin 1, presenilin enhancer 2) leading to atypical disease with diffuse involvement and many cysts
74
Systemic anticholinergics for treating hyperhidrosis?
Glycopyrrolate, propantheline, and oxybutynin
75
3 types of eccrine bromhidrosis?
■ Keratogenic: bacterial degradation of stratum corneum macerated by excess eccrine sweat
■ Metabolic: abnormal secretion of amino acids or breakdown products as seen in heritable metabolic disorders (e.g., phenylketonuria has mousy odor and maple syrup urine disease has sweet odor)
■ Exogenous: odorogenic compounds such as garlic, asparagus, curry, DMSO, penicillins
76
Red sweat?
clofazimine and rifampin
77
Yellow sweat?
lipofuscin
78
Blue/green sweat?
copper
79
Brown sweat?
dihydroxyacetone-containing self-tanning products
80
Fox-fordyce disease is also called?
Apocrine miliaria
81
Tx for Fox-fordyce disease?
Tretinoin, topical CS, TCIs, topical antibiotics, microwave ablation, and surgical excision; pregnancy can lead to improvement
82
Cutis rhomboidalis nuchae—solar elastosis variant affecting posterior neck with geometrically patterned leather-like wrinkled skin
83
Poikiloderma of Civatte: reticular reddish-brown telangiectatic patches on lateral neck (central submental region spared)
84
Same as Favre-Racouchot syndrome but clusters of white/yellow papules instead?
Colloid milium
85
Enzyme deficient in Porphyria cutanea tarda?
Uroporphyrinogen decarboxylase
86
Skin findings in PCT?
Skin fragility, vesicles, bullae, erosions, milia, scarring, hyperpigmentation, and hypertrichosis in photodistributed areas, Classic photo is hemorrhagic blisters on dorsal hands
87
Tx for PCT?
Avoid precipitating factors (alcohol & estrogen), sun avoidance, treat underlying conditions (if any), phlebotomy, low-dose (twice weekly) hydroxychloroquine
88
X-linked dominant protoporphyria is due to mutation in?
ALAS2 gene
Encodes 5-ALA synthase
↑↑ plasma protoporphyrin
89
Variegate porphyria enzyme?
Protoporphyrinogen oxidase
90
Hereditary coproporphyria deficiency?
Coproporphyrinogen III oxidase
91
Congenital erythropoietic porphyria (Gunther’s disease) deficiency?
Uroporphyrinogen III synthase
92
Erythrodontia seen with?
Congenital erythropoietic porphyria (Gunther’s disease)
93
Tx of Congenital erythropoietic porphyria (Gunther’s disease)?
strict photoprotection, hypertransfusions,
and iron chelation such as with deferoxamine; splenectomy may be considered, use of ascorbic acid and a-tocopherol has been advocated, ocular lubricants, and allogeneic bone marrow transplantation
94
Tx of Erythropoietic protoporphyria (ferrochelatase def.)?
strict photoprotection, oral b-carotene,
afamelanotide may be helpful in some patients, and hypertransfusion/plasmapheresis/exchange transfusion may be helpful in some patients; liver transplantation may be necessary with hepatic failure
95
Chondrodermatitis nodularis helicis chronica, Tender pink crusted papules on cartilaginous portions of ear helix and antihelix
96
Traumatic auricular hematoma; trauma to external ear → subperichondrial hematoma → cauliflower ear over time if not treated, seen with wrestlers
97
Black heel (talon noir): cluster of black pinpoint macules on posterior heel, Athletic trauma → rupture of supercial dermal vessels → hemoglobin in stratum corneum
98
Amyloidosis histology?
homogenous, eosinophilic, fissured
masses that stain with Congo red and have green birefringence with polarized light
Amyloid also stains positively with crystal violet, periodic acid–Schiff (PAS), and thioflavin T
99
Lichen amyloidosis
Rippled, hyperpigmented, pruritic papules/plaques on extensor surfaces (esp. shins), Seen in MEN 2A
100
Nodular amyloidosis, derived from Ig light chains, is assoc with?
Sjögren’s (25%), scleroderma and rheumatoid arthritis, Progression to systemic amyloidosis in 7%
101
Mediators of itch?
Histamine
Trypsin
Serotonin
Papain
Bradykinin
Substance P
VIP
Kallikrein
102
When is itch worse in CKD?
Worst at night and 2 days post-hemodialysis
103
Generalized pruritus is related to?
Hyperthyroidism vs. localized genital/perianal pruritus in diabetes mellitus
104
Drug-induced pruritus?
Opioids
Cocaine (formication)
Chloroquine
CTLA-4 or PD-1 inhibitors
BRAF inhibitors
EGFR inhibitors
Hydroxyethyl starch
105
Tx of choice for delusions of parasitosis?
Pimozide
QT prolongation on EKG, extra-pyramidal side effects, and drug-drug interactions
106
Burning mouth syndrome types?
Type I - better in morning and worsens through day
Type 2 - constant
Type 3 - sporadic periods of activity and remission
107
Notalgia paresthetica spinal level?
Medial scapular borders at T2–T6
108
Localized numbness, burning, tingling, allodynia, or pruritus of anterolateral thigh secondary to pressure on lateral femoral cutaneous nerve as it passes under the inguinal ligament, often seen in middle-aged obese males?
Meralgia paresthetica
A/w Obesity, pregnancy, prolonged sitting, tight clothing, heavy wallets in pant pockets
109
Trigeminal trophic syndrome
- Self-induced lesions of central face triggered by paresthesias/dysesthesias secondary to impingement or damage of sensory portion of trigeminal nerve
- Frequently occurs after treatment for trigeminal neuralgia w/ ablation of Gasserian ganglion
110
Neurodegenerative disease → defective lacrimation, absence of tongue papillae, taste disturbance and ↑ salivation, impaired regulation of temperature and bp,↓ pain sensation, absent tendon reflexes, hyperhidrosis, transient erythema of trunk, vomiting crises, and acrocyanosis of hands?
Familial dysautonomia/Riley-Day syndrome
AR inheritance of IKBKAP (locus 9q31)
111
Most common complication of parotidectomy?
Auriculotemporal nerve syndrome or Frey syndrome
Most common cause in children is nerve damage from forceps delivery, starts after starting solid foods
112
Punctate keratosis of the palmar creases, 1-5 mm small plugs in creases of palms or fingers primarily in African American patients
113
Kwashiorkor vs. Marasmus
Kwashiorkor: ↓ protein intake
♦Dyschromia
♦Hypopigmentation following trauma
♦“peeling, flaky paint” appearance
♦“Flag sign”
♦Compromised wound healing
♦Edema
Marasmus: ↓ energy/calorie intake
♦Thin, dry, pale, wrinkled, loose skin
♦Lanugo-like hair
♦Purpura
♦ Impaired hair and nail growth
♦“monkey facies”—↓ buccal fat pads
114
Excess Beta-carotene seen in?
Diabetes, nephrotic syndrome, and hypothyroidism, carrots, squash
115
Phrynoderma (keratotic follicular papules resembling toadskin), night blindness, xerophthalmia deficiency of?
Vitamin A
116
Oral-ocular-genital syndrome (cheilitis, angular stomatitis, seborrheic dermatitis-like rash, tongue atrophy/glossitis, genital and perinasal dermatitis) seen w/ deficiency of?
Vitamin B2 (riboflavin)
116
Selenium deficiency?
Hypopigmentation of skin, brittle hair, leukonychia, xerosis
117
Vitamin B3 (niacin) excess?
Flushing, pruritus, acanthosis nigricans
118
B3 or niacin deficiency is seen with?
Hartnup dz, alcoholism, carcinoid syndrome, isoniazid, 5-FU, azathioprine
119
Vitamin B6 (pyridoxine) def?
Periorificial seborrheic dermatitis-like rash, angular cheilitis, intertrigo, atrophic glossitis
120
Folic acid AKA?
Vitamin B9, Goat milk diet can predispose
121
Vitamin E excess causes?
Petechiae, ecchymoses
122
Infections that cause dystrophic calcification?
Onchocera volvulus and Taenia solium
123
Px in Takayasu's arteritis?
Purpura, Erythematous subcutaneous nodules, EN-like lesions, PG-like lesions and Raynaud's
124
Cutaneous fx of Type I (IgM, lymphopriliferative) cryoglobulinemia?
○Purpura, livedo reticularis, livedo racemosa, ulceration
○Cold-induced acrocyanosis of helices
○Raynaud phenomenon
124
Cutaneous fx of Type II and III cryoglobulinemia?
○Palpable purpura and urticarial lesions ○Systemic findings common
125
Livedoid vasculopathy (atrophie blanche)
Tx: ASA, Pentoxyfilline, Anticoagulation (rivaroxaban)
126
Schamberg’s: cayenne-pepper purpura on the lower extremities (esp. shin, ankles) that can extend; middle-aged to older adults
127
Purpura annularis telangiectodes of Majocchi: annular patches with punctate petechiae on trunk and lower extremity in adolescent/young-adult women
128
What's Levamisole-induced vasculitis?
Levamisole is an antihlmithic agent may be found in cocaine which ↑ its stimulant effects and bulk, purpura and necrosis of the earlobes (but also nose, cheek, extremities), LCV-like lesions, ecchymoses, and systemic vasculitis
129
Degos disease skin fx?
Crops of small erythematous papules that develop a central depression, ivory scar, peripheral erythema and surrounding telangiectasias (similar to atrophie blanche)
130
Livedo racemosa?
Cutaneous manifestation of Sneddon syndrome (=vasculopathy)
Livedo racemosa = larger branching and incomplete rings (vs. smaller complete rings of LR)
131
Erythromelalgia, plunging red, painful feet into ice cold water, genetic mutation (seen with type 2) is due to?
SCN9A mutations, Na+ channel subunit mutation ↓ sympathetic neuron activity, and ↑ pain receptor sensitivity
132
Pathophysiology of livedo reticularis?
secondary to vasospastic response to cold
and improves with heat; processes that → ↓ blood flow to and within skin or ↓ blood draining out of skin → deoxygenated blood in venous plexus → livedo appearance
133
Sarcoidosis + EN + hilar lymphadenopathy, is?
Lofgren's syndrome
134
Histo of Erythema nodosum?
* Septal panniculitis with thickening, fibrosis of septae
* Neutrophils seen particularly in early lesions
* Miescher’s microganulomas: small histiocytic aggregates surrounding a central stellate cleft
135
Bruised, tender plaques on lower extremities → ulceration/necrosis → oily discharge, is?
Alpha1-antitrypsin deficiency panniculitis
136
Anisotrichosis?
↑ variability in hair shaft size
137
Barrel-shaped spores?
Coccidioides immitis
138
Most reliable method for distinguishing between Trichophyton rubrum and T. mentagrophytes is?
Hair perforation test
Positive in T. mentagrophytes
139
What causes tinea nigra?
Phaeoannellomyces wernecki
140
Club shaped, and smooth walled, they grow singly or in clusters?
Epidermophyton floccosum
141
Pseudohyphae show?
1. Constrictions at the septae
2. Branching at the septae
3. Terminal cell is smaller than the others
142
Which human papillo viurs (HPV) type is implicated in papillomatosis cutis carcinoides di Gottron?
HPV 11
I.e. Bushcke-Lowenstein
143
Where do you see Gamma-Favre bodies?
Lymphogranuloma venereum (LGV)
144
Streptococcus Iniae has been shown to cause?
Hand cellulitis in fish handlers
145
Causative organism in Whitmore disease is?
Burkholderia pseudomallei
AKA Melioidosis
146
Mikulicz is the histologic hallmark of?
Rhinoscleroma
147
Treatment of choice for Oroya Fever?
Chloramphenicol
148
Xenopsylla cheopis transmits?
Murine typhus
149
All of the following are potential causes of a false positive RPR except:
1. Systemic Lupus Erythematosus (SLE)
2. Pregnancy
3. Malignant Melanoma
4. Lepromatous Leprosy
5. Malaria
3. Malignant Melanoma
150
The treatment of choice for Loiasis is?
Diethylcarbamazine (DEC)
151
Xenopsylla cheopis is the vector responsible for?
Endemic typhus
152
Erythema infectiosum is caused by a?
1. RNA virus
2. Herpes virus
3. Double stranded DNA virus
4. Single stranded DNA virus
5. None of these answers are correct
4. Single stranded DNA virus
153
Acrodermatitis chronica atrophicans is caused by?
1. Borrelia burgdorferi
2. Borrelia burgdorferi senso stricto
3. Borrelia garinii
4. Borrelia afzelii
4. Borrelia afzelii
154
Erythema gyratum repens is known to be associated with all of the following malignancies except:
1. Lung carcinoma
2. Breast carcinoma
3. Cervical carcinoma
4. Bladder carcinoma
5. Gastric carcinoma
5. Gastric carcinoma
155
A 55 year old patient presents with new onset brown macules on arms, legs, face and palms. She gives a 3 month history of diarrhea, abdominal cramps, weight loss and protein-losing enteropathy. The most likely diagnosis is:
1. Peutz-Jeghers syndrome
2. Cowden disease
3. Ulcerative colitis
4. Cronkhite-Canada syndrome
5. Plummer-Vinson syndrome
4. Cronkhite-Canada syndrome
156
A patient with congenital hypertrophy of retinal epithelium is most likely to have:
1. An autosomal dominant mutation in the MSH2 gene
2. Pheochromocytoma
3. Adenomatous polyposis
4. Tram-track calcifications on head radiograph
5. Peg-shaped teeth
3. Adenomatous polyposis
157
Tripe palms, “acanthosis palmaris,” are a cutaneous manifestation associated with which of the following malignancies?
1. Renal carcinoma
2. Lung carcinoma
3. Prostate carcinoma
4. Colon carcinoma
5. Pancreatic carcinoma
2. Lung carcinoma
158
Patients with Werners syndrome typically experience which of the following types of cardiac disease?
1. Hypertrophic cardiomyopathy
2. Aortic aneurysms
3. Premature atherosclerosis
4. Cardiomegaly
5. Mitral valve prolapse
3. Premature atherosclerosis
159
Which of the following statements about arthroconidia is correct?
1. Arthroconidia are formed by budding
2. Arthroconidia are formed by fragmentation of hyphae
3. Arthroconidia are thick-walled round cells
4. Arthroconidia are spores that are produced in a sac
2. Arthroconidia are formed by fragmentation of hyphae
160
Fungal culture reveals smooth, club-shaped macroconidia attached to hyphae in groups. No microconidida are seen. The organism is:
1. Microsporum canis
2. Epidermophyton floccosum
3. Trichophyton rubrum
4. Microsporum gypseum
2. Epidermophyton floccosum
161
Which disease is associated with increased cholesterol sulfate levels?
A. Lamellar ichtyosis
B. Non –bullous congenital Ichtyosiform erythroderma
C. X – linked Ichtyosis
D. Epidermolytic hyperkeratosis
C. X – linked Ichtyosis
162
One of the followings IS NOT FDA- approved indication for treatment of the following clinical diseases by interferones:
A. Condyloma acuminata
B. AIDS –associated Kaposi, s sarcoma
C. Melanoma
D. Actinic keratosis
D. Actinic keratosis
163
Dermoscopy showing Glomerular vessels is seen with?
Bowen's
164
The SITE of action of antifungal drugs class polyens is:
A. Blocks DNA synthesis
B. Disrupts mitotic spindle
C. Binds cell membrane sterols
D. Interferes with cell membrane synthesis via inhibition of squalene epoxidase
C. Binds cell membrane sterols
165
One of the following is a 4th generation cephalosporin?
A. Cefapime
B. Cephalexin
C. Cefaclor
D. Cefotaxime
E. Ceftriaxone
A. Cefapime
166
Suffix ximab in the nomenclature of biological agents indicates to What of the following ?
A. Blocker of interaction between interleukin- 1 and its receptor
B. Chimeric monoclonal antibody
C. Humanized monoclonal antibody
D. Human monoclonal antibody
B. Chimeric monoclonal antibody
167
Episodic angioedema, hypereosinophilia, weight gain and fever are characteristic of:
A. Kimura disease
B. Gleich syndrome
C. Melkersson – Rosenthal syndrome
D. Larva migrans
B. Gleich syndrome
168
Histology of AA?
169
Possible mutation in Central centrifugal cicatricial alopecia?
PADI3 mutation in some patients. A/w use of chemical relaxers, hot combs, traumatic hairstyles, pomades
170
"doll hairs," polytrichia = fusion of follicular infundibulae, seen in what alopecia?
Central centrifugal cicatricial alopecia
171
Graham-Little syndrome?
■ Associated with LPP
■ Scarring hair loss on scalp
■ Non-scarring hair loss of axilla and pubic areas
■ Keratosis pilaris-like spinous follicular papules on trunk
172
Neutrophilic alopecia's?
Folliculitis decalvans
Dissecting cellulitis
173
Mixed alopecia's?
Acne keloidalis
Erosive pustular dermatosis
Acne necrotica
174
Pseudoepitheliomatous epidermal hyperplasia is seen in?
Deep fungal infections
Leishmaniasis
Atypical mycobacteria
Tuberculosis verrucosa cutis
175
Histology of Acne keloidalis nuchae will show?
* Mixed (lymphoplasmacytic and neutrophilic) perifollicular inflammation at the isthmus and lower infundibulum
* Lamellar fibroplasia
* Loss of sebaceous glands
176
Most common causes of acquire generalized hypertrichosis?
Minoxidil, phenytoin, and cyclosporine
177
SPINK5 gene encodes?
Serine protease inhibitor LEKTI
178
“Cupid’s bow” upper lip; doughy skin; diffuse hypopigmentation (tyrosinase requires copper!) seen with?
Menkes hair disease, XLR, ATP7A (→ defective copper transport)
179
Features of Bazex-Dupre-Christol?
Pili torti, basal cell carcinomas, milia, follicular atrophoderma, hypohidrosis, hypotrichosis
180
Features of Netherton's?
1- Ichthyosis linearis circumflexa
2- Atopy
3- Hair abnormality: trichorrhexis invaginata, trichorrhexis nodosa
181
Pili bifurcate
182
Beau's lines are seen in?
Mechanical trauma
Chemotherapy
Stress on body/systemic illness
183
Mee's lines?
Arsenic or thallium
184
Koilonychia?
Normal in kids
Iron-deficiency, I,e. Plummer-Vinson
185
Subungual hyperkeratosis → thickened nail is termed?
Onychauxis
186
Red lunulae causes?
SLE, alopecia areata, rheumatoid arthritis, dermatomyositis, cardiac failure, CO poisoning
187
Lester iris seen with?
Nail-Patella syndrome
188
Causes of acute vs. chronic paronychia?
Acute - Staph aureus
Chronic - Candida
189
Subungual exostosis?
Subungual bony growth → nodule → nail plate elevation
190
Most common nail tumor?
Myxoid cysts - digital mucous cyst
191
Causes of fissured tongue?
A/w Melkersson-Rosenthal syndrome, Down syndrome, Cowden syndrome
192
Causes of Smooth tongue (atrophic glossitis)?
Vitamin deficiency (e.g., B1, B2, B6, B12, iron (e.g., Plummer-Vinson), folate), or other disorders (e.g., Sjögren’s syndrome)
193
Most common tumor of the oral cavity?
Fibroma
Pink smooth papule usually on buccal mucosa
Usually along bite line
194
Morsicatio buccarum?
White ragged, shredded surface changes of anterior buccal mucosa bilaterally due to habitual chewing of mucosa
195
White sponge nevus of buccal mucosa is due to mutations in?
Keratin 4 and 13
196
Gingival hypertrophy causes?
Phenytoin, nifedipine, cyclosporine
197
Orofacial granulomatosis seen in?
Melkersson-Rosenthal syndrome
Possibly also sarcoidosis and Crohn's
198
Enlargement, eversion, of lower lip with pinpoint erythema (inflammation of secretory ducts) + sticky mucoid film is termed?
Cheilitis glandularis
Adult men with h/o sun exposure ↑ SCC risk
199
Unilateral facial vitiligo, or poliois, with visual/hearing impairment on the same side is?
Alezzandrini syndrome
200
Chemical leukodermas?
Phenols, catechols, arsenic, PPD, merury, imiquimod, azelaic acid, methylphenidate patches, and sulfhydryls
201
Associations with hypomelanosis of Ito?
30% of patients also have CNS, musculoskeletal, or ophthalmologic abnormalities
202
Normal number of melanocytes with ↓ melanosomes is seen in?
Nevus depigmentosus
203
3 patterns of melasma?
Centrofacial, malar, and mandibular
Four types: epidermal (accentuated by wood's lamp), dermal, mixed, and indeterminate
↑ Melanin in all layers, ↑ melanophages, ↑ epidermal melanocytes
204
Prurigo pigmentosa tx?
Tetracyclines and dapsone
205
Mutation in Familial progressive hyperpigmentation?
Mutation of KIT ligand gene (KITLG)
206
Caput secundum and the midline?
its a 'cap', crosses the midline
207
Halo scalp ring
Occurs after prolonged labor, associated with caput secundum, may have necrosis or scarring
208
Nail fold capillaries in morphea?
Dilated capillary loops alternating w/ capillary drop out
209
Irregular telangiectasias w/ radiating vessels are seen with?
Hereditary hemorrhagic telangiectasia
210
Biomarker for skin and lung fibrosis
and pulmonary arterial HTN in scleroderma is?
CXCL4
211
Tx for pulmonary disease in scleroderma?
○ ILD: cyclophosphamide, rituximab, Mycophenalate mofetil*, HSCT, and adjuvant N-acetylcysteine
○ PAH: endothelin receptor antagonists (e.g., bosentan), PDE-5 inhibitors, prostacyclin analogs (e.g., iloprost), lung transplant
212
Tx option for digital ulcers in scleroderma?
IV iloprost, ASA/clopidogrel
213
Shulman syndrome is also called?
Eosinophilic fasciitis, px in fourth to sixth decades, unknown, but TGF-beta* is elevated
214
“Dry river bed” or “groove sign” = linear depressions of veins within indurated skin is seen in?
Eosinophilic fasciitis (Shulman syndrome)
215
What areas are spared in eosinophilic fasciitis?
Spares Face, hands & feet
Key feature = rapid onset of symmetric edema/ induration and pain in extremities in a/w peripheral eosinophilia
Lacks Raynaud phenomenon*
216
Pathogenesis of nephrogenic systemic fibrosis?
A result of gadolinium-containing contrast exposure (2-4 weeks) in the setting of acute kidney injury or severe chronic kidney disease (CKD) that leads to fibrosis of skin and internal organs
217
Features of of nephrogenic systemic fibrosis?
■ Insidious onset of symmetrically distributed, painless,
hyperpigmented and indurated “patterned plaques”
(reticular or polygonal morphology)
■ “pseudocellulite” or cobblestone appearance
■ Marked woody induration with peau d’orange changes
■ Puckering or linear banding due to focal areas of bound-down skin on proximal extremities
■ Dermal papules: browny to skin-colored papules or nodules with absent epidermal change
■ Scleral plaque (exam favorite!): white-yellow plaques w/dilated capillaries in patients < 45 yo (above this age, less specific)
218
Histology of nephrogenic systemic fibrosis is similar to?
Scleromyxedema, but fibrosis extends more deeply into fat and fascia
■ ↑ Collagen
■ ↑ Spindled fibrocytes (positive staining for CD34 and procollagen I)
■ ↑ Mucin (stains with Prussian blue)
219
Types of scleredema?
Type I: Preceded Strep pharyngitis
Type II: Assoc. w/ IgG-kappa
Type III: "Sceredema diabeticorum" IDDM
220
POEMS skin features?
Sclerotic skin changes favoring extremities, hyperpigmentation
Hypertrichosis
Hyperhidrosis
Digital clubbing
Leukonychia
221
Nephrogenic systemic fibrosis, is a form of sclerodermoid changes due to 'exogenous' substances, what are other types?
Toxic oil syndrome - Eruption, 'flu-like' sx's
Eosinophilia-myalgia syndrome - Fever, fatigue, severe myalgia's, due to ingestion of contaminated L-tryptophan
Polyvinyl chloride
Bleomycin - Pulmonary fibrosis*, Raynaud's*
Taxanes (docetaxel, paclitaxel) - Diffuse edema on legs
Nephrogenic systemic fibrosis
Sclerosis at injection sites - Vitamin K (Texier's disease), silicone or bleomycin injections
222
Keratotic crusted plug surrounding epithelial hyperplasia (“crab-claw”) grabbing pink elastic fibers in superficial dermis seen in?
Elastosis perforans serpiginosa
223
Primary (idiopathic) anetoderma types?
Jadassohn-Pellizzari - inflammatory
Schweninger-Buzzi - non-inflammatory
224
Atrophoderma vermiculatum?
Variant of follicular atrophoderma that is on face/cheeks exclusively
Seen in: ROMBO, Nocolau-Balus, and scrotal tongue
225
Pathogenesis of granuloma annulare?
Unknown etiology; most likely Th1-type delayed hypersensitivity reaction to a variety of triggers, trauma, isomorphic Koebner response, insect bites, tuberculin skin test, mycobacterial, viral infection, drugs, or UV radiation
226
Generalized granuloma annulare is associated with?
Hyperlipidemia (up to 45%), type I diabetes, HIV, thyroid disease, malignancy (clinical pattern atypical—e.g., palms and soles)
227
Most common histologic form of granuloma annulare?
Interstitial: dermal mucin between collagen fibers
228
Severe granuloma annulare tx?
Triple antibiotic regimens (minocycline, ofloxacin, and rifampin), TNF-a inhibitors, phototherapy, antimalarials, nicotinamide, isotretinoin, dapsone, pentoxiphylline, PDT
229
Multinucleated foreign body GCs than are typically seen in GA; phagocytosed elastic fibers within histiocytes and GCs (“elastophagocytosis”); no collagen alteration or lipid deposition, lacks mucin is seen in?
Annular elastolytic giant cell granuloma on face on sun exposed areas
230
Interstitial granulomatous dermatitis and arthritis (IGDA) and palisaded neutrophilic granulomatous dermatitis (PNGD)?
Types of granulomatous dermatitides existing on a spectrum assoc. w/ other systemic diseases like RA (in both) or SLE and ANCA vasculitides (more with PNGD)
231
Granulomatous drug eruption seen with?
CCBs, statins, TNF-a inhibitors
Months to years after drug initiation*
232
Dermoscopy of necrobiosis lipoidica?
Early lesions - comma-shaped vessels Older lesions - irregular arborizing vessels
233
Histological features of necrobiosis lipoidica?
"Square biopsy sign"
Plasma cells
Multinucleated giant cells
Horizontally arranged (“layered”) palisaded granulomatous inflammation
234
Ophthalmic manifestations in necrobiotic xanthogranuloma (periorbital region most commonly)?
50% have ophthalmic manifestations Ectropion, keratitis, uveitis, and proptosis
Majority also have endocardial involvement and hepatosplenomegaly common, strongly associated with IgG-kappa monoclonal gammopathy
235
Genetic predisposition for cutaneous Crohn's disease?
NOD2
Results in early-onset sarcoidosis called 'Blau' syndrome
+ defective microbial clearance, mucosal compromise or altered gut flora balance (dysbiosis) → exaggerated Th1 and Th17 response to gut flora → granulomatous lesions in gut and skin
236
Tx of cutaneous Crohn's?
First line: oral metronidazole, topical/intralesional steroids, and TCIs
Severe cases: oral steroids, sulfasalazine, MTX, MMF, cyclosporine, thalidomide, AZA, 6-MP, and TNF-a inhibitors
237
Sarcoidosis inflammatory pattern?
Upregulation of CD4+ Th1 cells
↑ IL-2, IFN-g, TNF-a, and monocyte chemotactic factor → monocytes leave circulation and enter peripheral tissues, including skin, where they form granulomas → granulomas have potential to result in end-organ dysfunction
238
Drug-induced sarcoid?
■ Hepatitis C patients on treatment (IFN-a, ribavirin)
■ HIV patients on HAART
■ Other meds: TNF-a inhibitors, vemurafenib, ipilimumab, and alemtuzumab
239
Sarcoid Variants?
Lupus pernio
Darier-Roussy
Löfgren syndrome
Heerfordt syndrome (“uveoparotid fever”)
Mikulicz syndrome
Blau syndrome
Drug-induced cutaneous sarcoid
240
Lupus pernio is associated with?
Chronic sarcoid lung (75%) and upper respiratory tract (50%) disease
Px w/ violaceous (rather than red-brown) papules coalescing into infiltrative plaques; nose, earlobes, cheeks = most common sites; “beaded” appearance along the nasal rim
241
Features of Darier-Roussy, variant of subcutaneous sarcoid?
Painless*, firm, deep-seated mobile nodules, Darier-Roussy is a form of subcutaneous sarcoid
242
Heerfordt syndrome vs. Mikulicz syndrome?
"Heerfordt syndrome" = Uveitis + parotid gland enlargement + fever + cranial nerve palsy
"Mikulicz syndrome" = enlargement of salivary, lacrimal, and parotid glands
243
Not routinely performed, but what is the Kveim-Siltzbach test?
Injecting suspension of sarcoidal spleen into the skin of a patient w/ sarcoidosis → sarcoidal granuloma at injection site
244
Histiocytoses stain positively for?
CD1a, S100, and Langerin (CD207) is most specific, stains Birbeck granules
245
Granuloma pattern with diffuse palisading and interstitial, horizontal “tiers” characteristic of?
Necrobiosis lipoidica
246
Red tattoos?
Mercuric sulfide, aka cinnabar px as Lichenoid dermatitis or pseduo-lymphoma
247
Sarcoidal or foreign body granulomas with needle- shaped or round crystals that are white and birefringent on polarized light?
Talc powder (hydrous magnesium silicate)
248
Persistent, soft brown papules in axilla can be due to?
Zirconium in antiperspirants
249
Dense neutrophilic infiltrate with
birefringent rhomboidal crystals seen with?
Zinc foreign body rxn
250
Non-Birefringent Foreign Body Granulomas?
Aluminum, beryllium, zirconium
251
Macrophage stain?
Positively w/ CD68 and HAM56
252
Langerhans cell histiocytosis mutation?
BRAF V600E activating mutation (50%–60%) → ↑ fibrosarcoma kinase activity → activation of RAS-RAF-MEK-ERK-MAP kinase pathway
253
Histology of Langerhans cell histiocytosis?
Dense proliferation of Langerhans cells (with reniform nuclei), regulatory T cells (FoxP3+, CD4+), and eosinophils in papillary dermis, with single and nested LCH cells in the epidermis
■ S100+, CD1a+, Langerin (CD207)+, CD68+
254
Tx for seborrheic-like LCH?
* Mild cutaneous disease:
Potent topical steroids, spontaneous resolution; diffuse disease: steroids + vinblastine
* Multisystem LCH: Vinblastine/prednisone for 1 year; other options: cladribine, cytarabine, clofarabine; emerging options: BRAF inhibitors, MEK inhibitors, sorafenib
255
Staining patterns for Non-LCH?
* All are CD68+, +/- Factor XIIIa+
* All are negative for Langerin
* S100 is negative in all (except ICH and Rosai-Dorfman)
* CD1a is negative in all (except ICH)
256
Historically known for intracytoplasmic “ comma-shaped/worm-like” bodies seen with?
Benign cephalic histiocytosis
257
Complement deficiencies associated with lupus?
C1q/r/s, C2, and C4
Screening test for complement levels? CH50
258
Drug-induced SCLE?
HCTZ, terbinafine, TNF-a inhibitors, PPI's, CCB's, griseofulvin
259
Biologics that can be used for SLE?
Belimumab [monoclonal human antibody that inactivates BLyS causing apoptosis and inhibition of B-cell maturation], anifrolumab
260
Jessner’s lymphocytic infiltrate of skin?
Photosensitive eruption, Possibly a variant of LE, appears on head, neck and upper back
261
Dermatomyositis with malignancy associated autoantibodies?
TIF1-g (p155) or NXP-2 (p140)
Ovarian + GI CA's*
Note: p140 in kids, indicates calcinosis cutis and contractures but not malignancy
262
Antisynthetase syndrome is characterized by?
○ Antisynthetase autoantibodies (Jo-1, PL-7, PL-12, OJ, EJ)
○Acute disease onset
○Mechanics hands*
○ILD*
○Constitutional symptoms
○Raynaud phenomenon
○Non-erosive arthritis
263
Schirmer test?
Whatman paper wick fold over
lower eye, if tear film migrates < 5 mm in 5 minutes = positive test
for Sjogren's
264
HLA in relapsing polychondritis?
HLA-DR4
Autoantibody titers against type II collagen correlate w/ disease activity
265
#1 cause of mortality in relapsing polycondritis?
Airway collapse and pneumonia
266
MAGIC syndrome?
MAGIC = Behçet’s disease + relapsing polychondritis
Mouth And Genital ulcers with
Inflamed Cartilage
267
Felty syndrome?
Seropositive RA characterized by neutropenia, splenomegaly, and refractory leg ulcers, resembling PG
↑ Risk of lymphomas/leukemias
Tx: G-CSF, +/- splenectomy
268
Bone feature characteristic of Adult Still's disease?
Carpal ankylosis - limited
range of motion with minimal pain
Life-threatening = Macrophage Activation Syndrome
269
Gluten-free safe foods?
rice, oats or corn
'ROCk'
270
DIF of 'chicken wire' appearance in PV versus PF?
PV — predominantly in lower epidermis (vs. PF)
PF — chicken-wire pattern evident in more superficial layers
271
Another name for dariers?
Keratosis follicularis
272
Drug-induced blistering?
Linear IgA bullous dermatosis: vancomycin
Pemphigus: penicillamine, captopril
Bullous pemphigoid: diuretics (esp furosemide)
273
Anti-Jo1 and Anti-PL7?
Anti-synthetase syndrome = myositis, mechanic’s hands, Raynaud's and severe ILD
