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Dermatology ANDREWS > Chapter 5 Atopic Dermatitis > Flashcards

Chapter 5 Atopic Dermatitis Flashcards

1
Q

Protein associated with atopic dermatitis

A

Filaggrin

Gene FLG, resides in epidermal differentiation complex, chrom1q21

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2
Q

Cytokine found high levels in AD skin

A

Thymic stromal lymphopoietin

(Produced by keratinocytes

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3
Q

T/F food allergy should be pursued only in children

A

True

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4
Q

Hallmark symptom of AD

A

Pruritus

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5
Q

Age and predilection of infantile AD, characteristic

A

2mos-2y
Face, extensor
Exudative, crust infiltration pustules

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6
Q

Age, predilection, characteristic of childhood AD

A

2-10y
Flexural areas eyelids facs neck
Less exudative, lichenified indurated plaques

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7
Q

Predilection, characteristic of adult/ado AD

A

Ado - flexural neck forehead periorbital
Adult - localized hand nipple eyelid
Erythematous scaly papular exudative or lichenified

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8
Q

T/F AD in ado or adults does not resolve over time

A

False

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9
Q

Reult of null mutations of FLG

A

Reduced natural moisturizing factor - caspase 14 process filaggrin during terminal keratinocyte differentiation to highly hydroscopic pyrrolidone carboxylic acid and urocanic acid

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10
Q

Result of subclinical dermatitis and abN delivery of lamellar body epidermal lipids (ceramide) to interstices of terminally differentiated keratinocytes

A

Increase transepidermal water loss TEWL

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11
Q

Name cutaneous stigmata of AD

A

Xerosis
Dennie-morgan folds
Hertoghe’s sign
Keratosis pilaris

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12
Q

Ophtha abN in AD

A

Anterior or posterior subcapsular cataracts

Keratoconus

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13
Q

Bacteria usually found in AD

A

S aureus

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14
Q

Common histology of AD

A

Hyperkeratosis acanthosis excoriation

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15
Q

Histological hallmark of eczema

A

Spongiosis with dermal perivascular lymphoid infiltrate and exocytosis

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16
Q

Most frequently affected site in ear eczema

A

External canal of ear

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17
Q

Mimicer of nipple eczema

A

Nevoid hyperkeratosis (unresponsive to cortecosteroids)

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18
Q

Most conmon occupational skin condition

A

Hand eczema

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19
Q

What is wet work

A

Skin in liquids or gloves for >2hrs per day or hand washing >20x per day

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20
Q

Primary lesion of acute pompholyx

A

Macroscopic deep seated multilocular vesicles resembling tapioca on sides of fingers

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21
Q

Predilection, primary lesion, histology of nummular eczema

A

Lower legs, dorsa of hands, extensor of arms
Discrete coin shaped erythematous edematous vesicular crusted patches
Acute to subacute spongiotic dermatitis

22
Q

Pathology of pruritic derm in elderly

A

Barrier failure due to loss of acidification of epidermis

Immune system skewed to Th2

23
Q

Gene mutation, skin lesion in X linked agammaglobulinemia

A

BTK gene

AD like, pyoderma gangrenosum

24
Q

Most common immunodeficiency state

A

Isolated IgA deficiency

25
Q

Drugs that induce IgA

A 
Phenytoin 
NSAIDs
Sulfasalazine
Cyclosporine 
Hydroxychloroquine
26
Q

Most common immunodef second to IgA def

27
Q

T/F anaphylactic reactions to IVIG asthma and AD are common in IgA Deficiency

28
Q

CD4/CD8 ratio of CVID

A

Less than 1 (compared to granulomas from sarcoidosis)

29
Q

Deficient cells in thymoma with immunodeficiency

A

B and pre-B cells (good syndrome)

30
Q

Chromosome in DiGeorge syndrome

31
Q

Gene mutation in IPEX syndrome

32
Q

Triad of SCID

A

Candidiasis of oropharynx and skin, intractable diarrhea, pneumonia

33
Q

Hematopoietic stem cell transplant in SCID should be done in what age

A

Before 3 and 1/2 months for optimum outcome

34
Q

Triad of wiskott-aldrich syndrome, gene mutation

A

Chronic eczematous dermatitis, increased susceptibility to bacterial infections (pyoderma or OM), thrombocytopenic purpura with small platelets
WASP gene - in hematopoietic cells, for reorganization of actin cytoskeleton in hematopoietic cells in response to external stimuli

35
Q

Gene mutation and function, initial prominent skin feature of ataxia telangectasia

A

ATM gene on chrom 11 - for DNA repair in double stranded breaks or for BDJ recombination of immunoglobulin and TCR genes, for maintenance of mitochondrial homeostasis

Progressive ocular and cutaneous telangectasia at 3-6y

36
Q

Gene mutation and function, defeciencies, skin lesion of WHIM syndrome

A

CXCR4 gene hyperactivity leading to retention of neutrophils in BM, basis of neutropenia and myelokathexis (increased apoptotic neutrophils in BM)
Common and genital warts

37
Q

DOCK8 deficiency associated with hyper IgE syndrome uniquely associated to which cutaneous viral infections?

A

HSV
Molluscum contagiosum
HPV

38
Q

Pathogenesis, skin lesion of CGD

A

Mutations in the gene that encode subunits of superoxide generating phagocyte NADPH oxidase responsible for respiratory burst in organism killing (no activation of phagocytic vacuoles)

Gingivostomatitis (aphthous like ulceration), seb derm of periauricular perinasal and parianal

39
Q

Diagnostic test of CGD

A

Low reduction of yellow nitroblue tetrazolium (NBT) to blue formazan in NBT test

40
Q

Gene mutation of LAD1 and LAD2 and LAD3

A

CD18 of beta 2 integrin family (ITGB2) - neutrophilia but with defect in migration
SLC35C1 - general decreased fucosylation of selectin ligands on leukocytes
FERMT3

41
Q

Gene mutation of autosomal dom and recessive hyper IgE

A

STAT3 - AD like eczematous derm, recurrent skin and lung infections, high IgE
DOCK8 - food allergies, warts, molluscum, herpes simplex, varicella

42
Q

Predilection of autosomal dom hyper IgE

A

Face scalp body

43
Q

Only autosom dom complement deficiency

A

C1 inhibitor

Hereditary angioedema

44
Q

C3 deficiency results in what infections

A

Encapsulted bacteria infections: s pyogenes, h influenzae, pneumococcus

45
Q

MASP2 deficiency results in what

A

Absent hemolytic activity by lectin pathway

Resembles SLE and increased pyogenic infection

46
Q

C2 deficiency results in?

A

SLE like syndromes, frequent infections, anaphylactoid purpura, dermatomyositis, vasculitis, cold urticaria

47
Q

Diagnostic test for complement deficiency

A

CH50 total hemolytic complement

48
Q

T/F acute GVHD is based on duration following transplantation not clinical presentation

49
Q

Skin lesions in acute GVHD

A

Erythematous morbilliform eruption of face and trunk which may become confluent and result in exfoliative erythroderma

50
Q

Symptoms of engraftment syndrome

A

Fever without infectious source, diarrhea, pulmo infiltrates with hypoxia, capillary syndrome with edema and weight gain

51
Q

Most common diagnostic feature of cGVHD

A

Lichen planus like eruption 3-5mos after grafting starting with hands and feet but becoming generalizes

52
Q

Prevention of posttransfusiob GVHD safely achieved by

A

Irradiating blood before transfusion

Dermatology ANDREWS (6 decks)

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