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Dermatology > Atopic Dermatitis > Flashcards

Atopic Dermatitis Flashcards

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Dermatology Board Prep flashcards
1
Q

Associated with atopic derm

A 
FLG mutations - more severe, also ass with allergic rhinitis & kp
RANTES
IL-13
Eotaxin
Beta subunit of Fc IgE R

Th2 downregulates FLG

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2
Q

% of AD that presents in first yr

A

50%

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3
Q

Pathogenesis of AD

A 
Activation of Th2 - 4,5,10,13
IL-10 inhibits delayed hypersensitivity
IL-4 downregulates IFN gamma
Staph superantigens decrease steroid responsiveness
Elevated prostaglandin E2
Elevated ach content of skin
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4
Q

MC Allergens in hair dressers

A

Glycerol monothioglycolate

Ammonium persulfate

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5
Q

Helpful in Ostomy dermatitis

A

Karaya powder

Cholestyramine

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6
Q

Oid oid disease

A

Barrier failure due to loss of acidification in elderly
Urticarial papules on the trunk/proximal ext that look like insect bites (clinically and histologically)
Elderly have loss of Th1
Associated with CCB

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7
Q

Autoimmune progesterone dermatitis

A

Urticarial and em type lesions most characteristic
Appear 5-7 d before menses, resolve a few days after menses - occurs during literal phase
OCP is treatment

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8
Q

X linked agammglobulinemia

A

Btk gene - essential for B cell development
Absent immunoglobulins and lymph nodes
Staph skin infections, also pseudomonas, h. Flu, strep
Hep b, rota, enterovirus common
Enterovirus may result in dermatomyositis-meningoencephalitis
Atopic derm-like dermatitis, pyoderma gangrenosum, RA like arthritis

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9
Q

MC immunodeficiency

A

IgA
Unknown gene, some found to have mutation in TNF R family
May develop CVID
Most asymptomatic > GI or respiratory > autoimmune dz
Incr risk of malignancy

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10
Q

CVID immune profile

A

Low IgA and IgG, 50% with low IgM
Predisposed to autoimmune do, gastric and lymphoreticular malignancies
Cutaneous granulomas

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11
Q

Hyper IgM (class switch recombination defects)

A

CD 40 and CD40 ligand deficiency, cytidine deaminase, uracil DNA glycosylase

Sinopulmonary, diarrhea, oral ulcers, recalcitrant HPV

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12
Q

MHC 1 expression reduced, decr cd8 but nl cd 4

Necrotizing granulomatous lesions on the nose or Lowe legs

A

TAP1 or 2 deficiency

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13
Q

MHC II deficiency

A

C2TA
RFX, RFXAP
RFSANK

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14
Q

ZAP-70 deficiency

A

AR
Enzyme required for normal intracellular T cell R signaling
Exfoliative erythroderma and eosinophilia
Nl cd4, decr cd8

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15
Q

Immune profile of anhidtrotoc ectodermal dysplasia with immunodeficiency

A

Lymphocytosis
Elevated cd3 and cd4 cells
Low NK cells

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16
Q

IPEX

A

FOXP3 - master TREG control gene
Immune dysfunction, polyendocrinopathy, enteropathy, xlinked
Ecz derm, psoriasiform scalp plaques, cheilitis, onychodystrophy
Staph sepsis

17
Q

SCID

A

Triad of candidiasis of skin/oropharynx, diarrhea, pneumonia
COD is usually viral infections
May see cutaneous granulomas in jak3 mutations
X linked MC

18
Q

WHIM

A

Warts, hypogammaglobulinemia, infections, myelokathis
CXCR4
Increased apoptotic neutrophils in the Bone marrow
Loss of CD27 memory B cells
Nl ab response but cannot maintain
Increased risk of ebv related lymphomas
Bronchiectasis

19
Q

CGD

A

XLinked MC, CYBB (leads to abscence of NADPH oxidase activity)
Gingivostomatitis, bad seb derm, subcorneal pustular eruptions

Female carriers may have raynauds, photosensitivity, oral ulceration, joint pain, DLE like lesions (no interface) but are ANA negative

20
Q

LAD

A

Type 1- common chain (CD18) of the b2 integrin family, gingivitis, periodontitis, oomphalitis, pg-like lesions

Type 2- FUCT1, absence of slex and other selectin log ands, MR

Type 3- KINDLIN3, recurrent info and bleeding tendency

21
Q

C’ component common to both pathways

A

C3

22
Q

Main mediator of opsonization

A

C3b

23
Q

Most common C’ deficiency

A

C2

Associated with SLE, dermatomyo, vasculitis, cold urticaria

C’ def associated SLE has early onset, photosensitivity, less renal dz and RO/LA in 2/3

24
Q

Properdin deficiency

A

X linked, predisposed to fulminant meningococcemia

25
Q

Engraftment syndrome

A

From neutrophil recovery
Fever, edema, pulm infiltrates, diarrhea
May have ocular involvement

Dermatology (28 decks)

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