Atopic Dermatitis Flashcards Preview

Dermatology > Atopic Dermatitis > Flashcards

Flashcards in Atopic Dermatitis Deck (25):
1

Associated with atopic derm

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

Th2 downregulates FLG

2

% of AD that presents in first yr

50%

3

Pathogenesis of AD

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

4

MC Allergens in hair dressers

Glycerol monothioglycolate
Ammonium persulfate

5

Helpful in Ostomy dermatitis

Karaya powder
Cholestyramine

6

Oid oid disease

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

7

Autoimmune progesterone dermatitis

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

8

X linked agammglobulinemia

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

9

MC immunodeficiency

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

10

CVID immune profile

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

11

Hyper IgM (class switch recombination defects)

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

Sinopulmonary, diarrhea, oral ulcers, recalcitrant HPV

12

MHC 1 expression reduced, decr cd8 but nl cd 4
Necrotizing granulomatous lesions on the nose or Lowe legs

TAP1 or 2 deficiency

13

MHC II deficiency

C2TA
RFX, RFXAP
RFSANK

14

ZAP-70 deficiency

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

15

Immune profile of anhidtrotoc ectodermal dysplasia with immunodeficiency

Lymphocytosis
Elevated cd3 and cd4 cells
Low NK cells

16

IPEX

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

17

SCID

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

18

WHIM

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

CGD

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

LAD

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

C' component common to both pathways

C3

22

Main mediator of opsonization

C3b

23

Most common C' deficiency

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

Properdin deficiency

X linked, predisposed to fulminant meningococcemia

25

Engraftment syndrome

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