General Dermatology Chapter 3 Flashcards
List the 6 hair disorders with + fragility?
Bubble hair
Monilethrix, “beaded hair”
Pili torti, “tortuous hair”
“Bamboo hair”
Trichorrhexis nodosa, “broomstick hair”
Trichothiodystrophy, “tiger tail hair”
Most common mutation identified in LCH lesions?
BRAF V600E
Factor XIIIa is found in?
NON-LCH conditions:
Juvenile xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, and Erdheim-Chester disease
LCH tx?
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.
Traumatic anserine folliculosis (TAF). Tx: Tretinoin 0.025%
PTCH1 mutations have been found in?
Gorlin syndrome, sporadic trichoepitheliomas, bladder carcinoma, and SCC of the esophagus
Accordion hand is a radiologic finding characteristic of?
Multicentric reticulohistiocytosis
Porphyria cutanea tarda can be a marker for?
Hereditary hemochromatosis
Costello syndrome have an increased risk of developing which?
A. SCC
B. Renal cell carcinoma
C. Rhabdomyosarcoma
D. Pneumothorax
E. Cardiac myxomas
C. Rhabdomyosarcoma
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. Trichphyton violacium
spindle lipoma’s develop on the?
posterior neck, upper back and shoulders
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
B. Angioid streaks
Which of the following is associated with seborrhea?
A. Scleroderma
B. Pachydermoperiostosis
C. Steatocytoma multiplex
D. Piezogenic pedal papules E. Pseudoxanthoma elasticum
B. Pachydermoperiostosis
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
D. Pediculosis pubis
Waldenstrom hypergamma globulinemic purpura is most frequently associated with?
A. Rheumatoid arthritis
B. Lupus erythematosus
C. Lymphoma
D. Multiple myeloma
E. Sjogren’s syndrome
E. Sjogren’s syndrome
What disorders are associated with IgA gammopathy?
Sweet’s syndrome
Pyoderma gangrenosum
IgA pemphigus and subcorneal
pustular dermatosis
Erythema elevatum diutinum
POEMS syndrome
What disorders are associated with IgM gammopathy?
Schnitzler’s syndrome
Waldenström macroglobulinemia
Cryoglobulinemia
IgG-k is associated with?
Necrobiotic xanthogranuloma
Scleredema
IgG-lamda is associated with?
Scleromyxedema
Secondary eruptive xanthomas due to what drugs?
Oral retinoids, protease inhibitors, olanzapine, estrogen replacement
Tuberous xanthomas mainly occur on?
Elbows and knees; a/w type II and III
Firm nodules on Achilles tendon are?
Tendinous xanthomas seen in type II hyperlipidemia
Dysbetalipoproteinemia assoc. with Tuberoeruptive, tuberous, plane, palmar creases?
Type III
Histology of xanthomas?
Foam cells, macrophages w/ lipidized cytoplasm, in dermis
Preformed mediators?
“Preformed HHP”
Heparin
Histamine
Proteases
Urticaria include immune complex deposition, urticarial vasculitis, and what other meds?
NSAIDs, radiocontrast media, polymyxin B, ACE inhibitors (due to ↑ bradykinin), and dietary pseudoallergens
Most common drug causing urticaria?
b-lactams
Blanched vasoconstricted halos
around pink wheal
○ Responds better to propranolol than to antihistamines
○ Can reproduce lesions by intradermal
norepinephrine injections
Aquagenic urticaria can be commonly seen with?
cystic fibrosis
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
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
↓ C1q levels?
seen in AAE only!
↓ C1 inh in type I HAE*
C1 inh deficiency tx?
Oral danazol is ToC for prophylaxis and C1 inh concentrate is ToC for acute attacks
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%)
For classic sweet’s gender and location predilection?
F > M, 3:1
Favors head, neck and upper extremities
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)
Lab abnormalities in Sweet’s?
■ ↑ ESR/CRP (90%)
■ Leukocytosis: neutrophilia
w/ ↑ band forms “left shift”
Triggers for sweet’s?
Infections: streptococcus, yersiniosis; HIV, hepatitis C
Drugs: G-CSF, GM-CSF, ATRA, TMP-SMX, minocycline, OCPs
Cancer: AML
Histiocytoid Sweet’s will show infiltrate of?
Deep dermal infiltrate of CD163+ macrophages
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
Pyoderma gangrenosum in kids?
on head and anogenital region
a/w IBD or leukaemia
Bullous PG?
More superficial, less destructive than classic PG, More widespread, a/w hematologic malignancy (AML,
CML, MDS, polycythemia vera)
Painless cribriform ulcers w/
verrucous growths on trunk of PG?
Vegetative PG
What’s the cause of Behçet’s disease?
Multifactorial (infectious trigger?); circulating immune complexes and neutrophil dysregulation → vascular injury 1st common sx: aphthous stomatitis
Behcet’s number cause of mortality?
Ocular (90%), including blindness
MAGIC syndrome?
MAGIC syndrome = features of Behçet’s + relapsing polychondritis
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%)
Neoplastic Hypereosinophilic syndrome fusion gene?
FIP1L1-PDGFRA fusion gene, tx with Imatinib if fusion gene present
Hypereosinophilic syndrome die from?
Congestive heart failure, they need regular echocardiograms
Immune reaction to antigen (e.g., tinea pedis, other dermatophyte infections, other fungi, viruses, parasites), drug, pregnancy, malignancies?
Erythema annulare centrifugum
Mild spongiosis, focal parakeratosis, and perivascular lymphohistiocytic infiltration, which is tight and dense “coat sleeve” is seen in?
Erythema annulare centrifugum
Agar for Borrelia?
Barbour-Stoenner-Kelly medium
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
Cutibacterium acnes activates TLR-2 on macrophages then activates what part of the inflammasome?
NLRP3 of neutrophils
Effect of tretrinoin on acne?
downregulate TLR-2 expression
Severe eruptive nodulocystic acne, without systemic manifestations (vs. acne fulminans)?
Acne conglobata
Common sites for neonatal acne?
Cheeks and nasal bridge
Infantile acne tx?
Topical retinoids, topical antibiotics, or in moderate-severe cases azithromycin or isotretinoin
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
SAPHO (Chronic recurrent multifocal osteomyelitis) stands for?
Synovitis
Acne
Pustulosis
Hyperostosis
Osteitis
PAPA syndrome AD mutation in?
CD2-binding protein 1 (CD2BP1; aka PSTPIP1)
“bull’s head” sign may be seen on X-ray?
SAPHO (Chronic recurrent multifocal osteomyelitis)
HAIR-AN?
Hyper Androgenism
Insulin Resistance
Acanthosis Nigricans
Unique subtype of PCOS
Mutation in fibroblast growth factor receptor 2, FGFR2?
Apert syndrome (Acrocephalosyndactyly)
Molecules inflamed in rosacea?
Kallikrein 5, TPRV, cathelicidin LL-37, and TLR2 are upregulated
Haber’s syndrome?
Genodermatosis w/ rosacea-like eruption and verrucous lesions
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
Gram-negative folliculitis is seen in?
Patients receiving prolonged antibiotic treatment (esp. tetracyclines)
Eosinophilic pustular folliculitis (Ofuji’s disease) + Peripheral eosinophilia; Tx: oral indomethacin
Tx of Trichodysplasia spinulosa?
Topical cidofivir, tazarotene gel, oral
valganciclovir
Tx of Pseudofolliculitis barbae?
Bunch of tiny curled hairs
Tx: Stop shaving; laser hair removal,
Follicular occlusion tetrad?
Acne conglobata
Hidradenitis suppurativa
Dissecting cellulitis of the scalp
Pilonidal cyst
HS mutation?
“Gamma-secretase complex” (nicastrin, presenilin 1, presenilin enhancer 2) leading to atypical disease with diffuse involvement and many cysts
Systemic anticholinergics for treating hyperhidrosis?
Glycopyrrolate, propantheline, and oxybutynin
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
Red sweat?
clofazimine and rifampin
Yellow sweat?
lipofuscin
Blue/green sweat?
copper
Brown sweat?
dihydroxyacetone-containing self-tanning products
Fox-fordyce disease is also called?
Apocrine miliaria
Tx for Fox-fordyce disease?
Tretinoin, topical CS, TCIs, topical antibiotics, microwave ablation, and surgical excision; pregnancy can lead to improvement
Cutis rhomboidalis nuchae—solar elastosis variant affecting posterior neck with geometrically patterned leather-like wrinkled skin
Poikiloderma of Civatte: reticular reddish-brown telangiectatic patches on lateral neck (central submental region spared)
Same as Favre-Racouchot syndrome but clusters of white/yellow papules instead?
Colloid milium
Enzyme deficient in Porphyria cutanea tarda?
Uroporphyrinogen decarboxylase
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
Tx for PCT?
Avoid precipitating factors (alcohol & estrogen), sun avoidance, treat underlying conditions (if any), phlebotomy, low-dose (twice weekly) hydroxychloroquine
X-linked dominant protoporphyria is due to mutation in?
ALAS2 gene
Encodes 5-ALA synthase
↑↑ plasma protoporphyrin
Variegate porphyria enzyme?
Protoporphyrinogen oxidase
Hereditary coproporphyria deficiency?
Coproporphyrinogen III oxidase
Congenital erythropoietic porphyria (Gunther’s disease) deficiency?
Uroporphyrinogen III synthase
Erythrodontia seen with?
Congenital erythropoietic porphyria (Gunther’s disease)
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
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
Chondrodermatitis nodularis helicis chronica, Tender pink crusted papules on cartilaginous portions of ear helix and antihelix
Traumatic auricular hematoma; trauma to external ear → subperichondrial hematoma → cauliflower ear over time if not treated, seen with wrestlers
Black heel (talon noir): cluster of black pinpoint macules on posterior heel, Athletic trauma → rupture of supercial dermal vessels → hemoglobin in stratum corneum
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
Lichen amyloidosis
Rippled, hyperpigmented, pruritic papules/plaques on extensor surfaces (esp. shins), Seen in MEN 2A
Nodular amyloidosis, derived from Ig light chains, is assoc with?
Sjögren’s (25%), scleroderma and rheumatoid arthritis, Progression to systemic amyloidosis in 7%
Mediators of itch?
Histamine
Trypsin
Serotonin
Papain
Bradykinin
Substance P
VIP
Kallikrein
When is itch worse in CKD?
Worst at night and 2 days post-hemodialysis
Generalized pruritus is related to?
Hyperthyroidism vs. localized genital/perianal pruritus in diabetes mellitus
Drug-induced pruritus?
Opioids
Cocaine (formication)
Chloroquine
CTLA-4 or PD-1 inhibitors
BRAF inhibitors
EGFR inhibitors
Hydroxyethyl starch
Tx of choice for delusions of parasitosis?
Pimozide
QT prolongation on EKG, extra-pyramidal side effects, and drug-drug interactions
Burning mouth syndrome types?
Type I - better in morning and worsens through day
Type 2 - constant
Type 3 - sporadic periods of activity and remission
Notalgia paresthetica spinal level?
Medial scapular borders at T2–T6
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
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
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)