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91 Cards in this Set
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- Back
- 3rd side (hint)
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Where do skin-associated lymphocytes reside (epidermis vs. dermis)?
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2% in epiderimis= CD8+ memory
98% in dermis |
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What are the 3 layers of the skin's basement membrane/
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1. Lamina Lucida- contains exracellualar part of hemidesmisomes
2. Lamina Densa- collagen IV adn laminin network 3. Sublamina Densa- anchoring filaments, Collagen VII |
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What markers are found on dermal dendritic cells?
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Factor XIIIa, DC-SIGN/CD209+, CD1b, CD1c. Present antigen
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What markers are found on dermal T lymphocytes?
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CD4 and CD8+
memory CLA-1 |
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Where are typtase chymase mast cells (MCtc)found?
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skin, conjunctiva, heart, intestinal mucosa.
Have CD88 R for C5a |
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Where are typtase mast cells found (MCt)?
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alveolar wall, respiratory epithelium, small intestine mucosa
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What is fillagrin? What diseases have mutated fillagrin?
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Filaggrin- matrix protein involved in bonding of keratin filaments.
Mutations- eczema and icthyosis vulgaris |
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Where should you biopsy if you suspect an immunobullous disease?
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2 biopsies
1. shave biopsy of an intact vesicle (H and E) 2. perilesional tissue for immunofluorescence |
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Pemphigus Vulgaris
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) Flaccid bullae, crusting, Nikolsky +. Affects scalp, chest, intertrigenous, oral mucosa
2)IgG to desmoglein 3>1 3)Epidermal IgG and C3, suprabasal layers |
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Pemphigus Folliaceous
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1)Superficial bullae, crusting, +Nikolsky
2)IgG to desmoglein 1 3)Epidermal IgG and C3 staining of granular layer |
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Paraneoplastic Pemphigus
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1)Flaccid bullae, mucosa, +Nikolsky
2)IgG to plakin proteins and desmoglein 1 and 3 3)Epidermal IgG and C3 cell surface and basement membrane |
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IgA Pemphigus
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1)flaccid lakes of pus
2)IgA to desmoglein 1 and 3 3)Epidermal IgA cell surface |
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Bullous Pemphigoid
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) Tense bullae, few with Nikolski (10%)
2)IgG to BP180 and BP 230 3)Linear basement membrane IgG and C3 |
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Cicaricial Pemphigoid
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) No bullae, +erosions, scarring
2)IgG to BP180 and laminins 3)Linear basement membrane IgG and C3 |
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Herpes Gestationalis
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) Tense bullae, 2nd trimester, pruritus
2)IgG to BP180 3)Linear basement membrane C3 |
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Epidermolysis Bullosa Acquisita
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) Tense bullae, erosions, scarring
2)IgG to Collagen VII 3)Linear basement membrane IgG and C3 |
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Linear IgA Bullous Dermatosis
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence? |
1) Tense bullae, oral
2)IgG to LABD97, LAD-1 3)Linear basement membrane IgA |
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Dermatitis Herpeteformis
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence |
1)Small bullae on elbows, knees, pruritic
2)IgA ab to TTG in epidermis 3)Granular basement membrane zone IgA with stippling in dermal papillae |
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Bullous Lupus Erythematosus
1) Presentation? 2) Serum Autoantibodies? 3) Immunofluorescence |
1)Tense bullae, photo distribution
2)IgG to Collagen VII 3)Linear basement membrand IgG, granular IgM, and C3 |
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SLE
1)Indirect Immunoflouresence? 2)Direct Immunofluorescence of Tissue? 3)Nuclear antigens targeted? |
1)peripheral, nucleolar, speckled
2)IgG, IgM , and/or IgA with C3 deposit in basement membrane 3)nDNA, dsDNA, ssDNA, histones, nucleolar RNA, Smith, U1-snRNP, HMG-17 |
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Discoid Lupus Erythematosis
1)Indirect Immunoflouresence? 2)Direct Immunofluorescence of Tissue? 3)Nuclear antigens targeted? |
1)No circulating Ab
2)IgG, IgM , and/or IgA with C3 deposit in basement membrane 3)None |
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Subacute Cutaneous Lupus Erythematosis
1)Indirect Immunoflouresence? 2)Direct Immunofluorescence of Tissue? 3)Nuclear antigens targeted? |
1)Speckled
2)With or without immune deposit in basement membrane 3)SS-A (ro) and SS-B (la) |
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Neonatal Lupus Erythematosis
1)Indirect Immunoflouresence? 2)Direct Immunofluorescence of Tissue? 3)Nuclear antigens targeted? |
1)Speckled
2)Granular IgG (transplacental) at basement membrane 3)SS-A (ro) and SS-B (la |
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Drug Induced Lupus Erythematosis
1)Indirect Immunoflouresence? 2)Direct Immunofluorescence of Tissue? 3)Nuclear antigens targeted? |
1)Homogeneous
2)Granular immune deposits at BMZ 3)Histones |
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Cutaneous Scleroderma
1)Indirect Immunofluorescence (serum)? 2)Direct Immunoflourescence (tissue)? 3)Targeted Nuclear Antigens 3) |
1)Peripheral
2)nothing characteristic 3)Scl-70, SS-A, SS-B |
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Limited Scleroderma
1)Indirect Immunofluorescence (serum)? 2)Direct Immunoflourescence (tissue)? 3)Targeted Nuclear Antigens |
1)Centromere
2)Nothing Characteristic 3)centromere, Scl-70, U1-snRNP, HMG-17 |
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Progressive Systemic Sclerosis
1)Indirect Immunofluorescence (serum)? 2)Direct Immunoflourescence (tissue)? 3)Targeted Nuclear Antigens |
1)Nuclear, speckled
2)nothing characteristic 3)Scl-70, U1/U3-snRNP, fibrillarin, RNA-pol I, II and III |
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Dermatomysitis and Polymyositis
1)Indirect Immunofluorescence (serum)? 2)Direct Immunoflourescence (tissue)? 3)Targeted Nuclear Antigens |
1)speckled, nucleolar
2)nothing characteristic 3)Jo-1, PM-Scl, Mi-2, U1-snRNP, SS-A, SS-B |
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Sjogrens
1)Indirect Immunofluorescence (serum)? 2)Direct Immunoflourescence (tissue)? 3)Targeted Nuclear Antigens |
1)fine speckled
2)nothing characteristic 3)SS-A, SS-B, histones, U1-snRNP |
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Mixed CT Disease
1)Indirect Immunofluorescence/ 2)Direct Immunofluorescence? 3)Targeted Nuclear Antigens? |
1)Speckled
2)nothing characterisitic 3)U1-snRNP, PM-scl |
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What are defensins?
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Anti-bacterial peptides (also 5% of proteins in neutrophilic granules). Increased synthesis in response to IL-1, TNF secreted by macros
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What disease should you consider when an adult presents with new onset dermatitis without a history of asthma/allergies/eczema as a child?
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Cutaneous T cell Lymphoma.
Do a skin biopsy! |
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What receptor is upregulated on Langerhan's cells, inflammatory dendritic cells in AD?
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FCeRI
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What chemokines are specific for AD?
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CTAK, CCL27, TARC
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A decrease/absence of which antimicrobial peptides is seen in AD?
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Human B defensin 2 and 3
Cathelicidin LL-37 HBD 3 deficiency leads to staph aureus infection |
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What TLR defect leads to severe AD with frequent defects?
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TLR 2
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What cytokines are involved in acute and chronic AD?
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Acute= IL-4 and IL-13
Chronic= IL-5, IL-12, INFg |
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Where is AD typically distrubuted in kids >2yrs and adults?
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flexural areas of hands, feet, face, antecubital/popliteal fossa
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Where is AD typically distrubuted in kids <2yrs?
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Extensor surfaces, neck, trunk, and face
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What are the major diagnostic criteria for AD?
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pruritus, chronic, relapsing, family history of atopy
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What aeroallergens trigger AD?
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dust mite, animal dander, weeds, mold
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What are Tranta's dots associated with?
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AKC. Activity parallels that of AD skin flares.
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What type of cataracts is associated with AKC?
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anterior subcapsular
Posterior= glucocorticoids |
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What foods account for nearly 90% of eczema food assoc flares?
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milk, egg, soy, wheat, peanut, TN, fish
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What else triggers AD flares (other than food)?
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dust mites, staph aureus super antigen, autoantigens, toxins on skin
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What antibody is responsibble for chonic autoimmune urticaria?
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IgG to alpha subunit of FCeRI or less commonly an anti-IgE ab
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What mediators are released after binding antigen to IgE bound on basophils and mast cells?
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mast cells= histamine , prostaglandin D, LTC4, LTD4, PAF
Complement= C3a, C4a, C5a, Bradykinin |
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What causes hives?
ITCHING MAPS |
I- infection; T- transfusions; C-Chronic idiopathic; H- hereditary (familial cold, amyloidosis, Muckle-wells); I- inhaling or contact allergens; N= NSAIDS or Neoplasms
G= Gut (foods, additives); M- Mastocytosis; A- autoimmune; P- physical urticarias; S- SLE, collagen vascular diseases |
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In which cold urticaria syndromes is the ice cube test negative?
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cold induced cholinergic urticaria,
systemic cold urticaria, cold dependent dermatographism |
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What is the key difference between exercise induced anaphylaxis and cholinergic urticaria?
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EIA will not react with passive heating.
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Hypocomplementemic urticarial vasculitis syndrome (HUVS) features?
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urticaria, low complement (C3, C4), angioedema, obstructive lung disease, uveitis, and episcleritis. +anti-C1q antibodies, low C1q, increased ESR
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What are the predominant histiologic featues of allergic contact derm?
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Lymphocytic infiltration, spongiosis
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What are the 10 most common contact allergens in the US?
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nickel, balsam of Peru, neomycin, cobalt, fragrance mix, potassium dichromate, bacitracin, thimerosal, formaldehyde, glutaraldehyde
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Where is potassium dichromate found?
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stainless steel, chrome plating, tanned leather
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Where are chromates found?
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textile, leather tanners, wet cement workers
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Where is cobalt dichloride found?
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dental implants, artificial joints, engines, rockets
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What causes toxicodendron dermatitis (poison ivy/oak)?
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oleoresin (sap and crushed part of plant)
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If you are highly susceptible to poison ivy what fruit peel exposure may cause cross reactivity?
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mango peels
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What is the most common cause of contact hypersensitivity in hair dressers? clue-- also in henna tatoos
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Paraphenylenediamine
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What should you use for patch testing with topical corticosteroids?
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budesonide
tixcortol pivalate 1% |
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Name important skin targets in Immunologic Skin Diseases.
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BP180
BP230 Desmoglein 1 Desmoglein 3 Type VII collagen (anchoring fibrils) |
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How do you treat Pemphigus Vulgaris?
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Prednisone,
+/- other immunosupressives |
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What serum antibody is + in Pemphigus Vulgaris?
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IgG to Desmoglein 3 and/or desmosome
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What does tissue immunofluoresence show in pemphigus vulgaris?
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IgG and C3 to epidermal cell surface
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What serum antibody is + in Pemphigus Foliaceous?
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IgA to desmoglein 1 and/or desmosome
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What does tissue immunofluoresence show in pemphigus vulgaris?
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IgA on epithelial surface
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What are the target antigens in bullous pemphigoid?
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BP180, BP230, hemidesmisome, lamina lucida
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What is seen on tissue immunofluorescence in herpes gestationalis?
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C3 in linear basement membrane zone.
C3 target? |
BP180 (BP 230, hemidesmosome, lamina lucida)
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What is the target antigen/protein in dermatitis herpeteformis?
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epidermal TTG
what serum autoanitbody? |
IgA (stippling in dermal papillae)
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How many grams of a steroid ointment cover the entire body of an average adult?
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30gms
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List adverse effects of steroid ointments?
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thinning, telangiectasia, bruising, hypopigment, acne, striae, and secondary infections.
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What is the black box on topical calcineurin inhib? What age is indicated?
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>2yr old
risk of cutaneous lymphome given lack of long term safety data. |
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List low dose topical steroids.
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hydrocortisone acetate
desonide alclometasone dipropionate |
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List medium dose topical steroids.
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betamethasone valerate
fluticasone propionate hydrocortisone butyrate hydrocortisone valerate mometasone furoate triamcinolone acetonide |
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List high dose topical steroids.
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betamethasone dipropionate
clobetasole propionate diflorasone diacetate halobetasol |
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Name the ophthalmic drops that are both antihistamine and mast cell stabilizers.
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olopatadine
ketotifen azelastine epinastine |
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What is transglutaminase?
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cross links keritinized epithelial cells in the epidermis
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What are desmosomes?
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contain e-cadherins, attach adjacent epithelial cells
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What are hemidesmosomes?
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use integrins for adhesion (not cadherins), attach epidermis to dermis
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What are fillagrins?
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filament associated proteins that bind keratin fibers
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What are the layers of the epidermis?
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Stratum Basale
Statum Spinosum Stratum Granulosum Stratum Lucidum Stratum Corneum ++Before Signaling Get Legal Counsel |
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What are the different types of epithelial cells?
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Simple/statified squamous
Simple/Stratified cuboidal Simple columnar Pseudostratified columnar Transitional |
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What endothelial receptor does Rhinovirus bind?
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ICAM-1
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What endothelial receptor does Adenovirus bind?
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ICAM-1
CD80 CD86 |
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What endothelial receptor does Influenza bind?
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Glycans
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In pemphigus vulgaris, what do you see on direct immunofluorescence? What is the target antigen? Location?
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Direct IF= Intercellular IgG, IgM to desmoglein 3 in Desmosome
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In pemphigus folaceous, what do you see on direct immunofluorescence? What is the target antigen? Location?
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Direct IF= Intercellular IgG to desmoglein 1 in Desmosome
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In bullous pemphigoid, what do you see on direct immunofluorescence? What is the target antigen? Location?
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Direct IF: Linear IgG to basement membrane BP180, BP 230 in hemidesmosome
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In pemphigoid gestationalis, what do you see on direct immunofluorescence? What is the target antigen? Location?
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Direct IF: Linear IgG to basement membrane BP180, BP 230 in hemidesmosome
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In Dermatitis Herpetiformis, what do you seen on direct immunofluorescence? What is the target antigen? Location?
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Direct IF: Granular IgA in dermis to Transglutaminase in subepidermis
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In Linear IgA dermatosis, what do you seen on direct immunofluorescence? What is the target antigen? Location?
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Direct IF: Linear IgA at basement membrane to BP 180 in hemidesmosome
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