Atopic Dermatitis Flashcards
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
% of AD that presents in first yr
50%
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
MC Allergens in hair dressers
Glycerol monothioglycolate
Ammonium persulfate
Helpful in Ostomy dermatitis
Karaya powder
Cholestyramine
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
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
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
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
CVID immune profile
Low IgA and IgG, 50% with low IgM
Predisposed to autoimmune do, gastric and lymphoreticular malignancies
Cutaneous granulomas
Hyper IgM (class switch recombination defects)
CD 40 and CD40 ligand deficiency, cytidine deaminase, uracil DNA glycosylase
Sinopulmonary, diarrhea, oral ulcers, recalcitrant HPV
MHC 1 expression reduced, decr cd8 but nl cd 4
Necrotizing granulomatous lesions on the nose or Lowe legs
TAP1 or 2 deficiency
MHC II deficiency
C2TA
RFX, RFXAP
RFSANK
ZAP-70 deficiency
AR
Enzyme required for normal intracellular T cell R signaling
Exfoliative erythroderma and eosinophilia
Nl cd4, decr cd8
Immune profile of anhidtrotoc ectodermal dysplasia with immunodeficiency
Lymphocytosis
Elevated cd3 and cd4 cells
Low NK cells
IPEX
FOXP3 - master TREG control gene
Immune dysfunction, polyendocrinopathy, enteropathy, xlinked
Ecz derm, psoriasiform scalp plaques, cheilitis, onychodystrophy
Staph sepsis
SCID
Triad of candidiasis of skin/oropharynx, diarrhea, pneumonia
COD is usually viral infections
May see cutaneous granulomas in jak3 mutations
X linked MC
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
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
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
C’ component common to both pathways
C3
Main mediator of opsonization
C3b
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
Properdin deficiency
X linked, predisposed to fulminant meningococcemia
