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132 Cards in this Set
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MC autoimmune blistering disease
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Bullous pemphigoid
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Pephigus Foliaceus
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all ages
red crusting blisters chest, under eyes few, fragile called pemphigus erythematous when w SLE drugs: PCN-D, captopril IgG and C3 (upper epi) |
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Pemphigus
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elderly
oral esophageal erosion flaccid blisters NO itch NIKOLSKY's NO FACE IgG (mid epi) rare paraneoplastic pemphigus |
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pemphigus erythematous
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pemphigus foliaceus with SLE
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Cicatrical Pemphigoid
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elderly
oral blisters/erosions EYES conjunctival fibrosis with blindenss may follow SJS few blisers face, neck, chest NO BODY tense blisters, heal with scars ITCH IgG and C3 (DEJ) |
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Pemphigoid
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elderly
ITCH URTICARIAL plaques PALMS and SOLES Nikolsky's Tense blisters IgG and C3 (DEJ) |
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Dermatitis Herpetiformis
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middle age
SEVERE ITCH burning vesicles Gluten-senstive enteropathy Serum IgA-Ema IgA Fibrin dermis rare SMALL BOWEL LYMPHOMA |
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Subcorneal Blisters
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Candida Albicans
Impetigo Milaria crystallina SSS Subcorneal pustular dermatosis "MISS Canada" |
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Granular Layer Blisters
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bullous Ichthyosiform erythroderma
Pemphigus foliaceus Pemphigus erythematous |
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Spinous Layer Blisters (upper and mid epi)
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upper and mid epidermis
Dermatophyte Dyshyrosis Eczema Friction Insect bite and scabies Miliaria rubra Viral (HSV, zoster) "MF DIVE" |
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Spinous Layer (lower and suprabasal epi)
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Benign familial chronic pemphigus
Keratosis follicularis Pemphigus vulgaris transient acantholytic dermatosis "PT BK" |
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Basal Cell Blisters
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EM
Epidermolysis bullosa simplex Fixed drug Kerosene necrosis LP TEN "FEEL TEN" |
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Laminal Lucida Blisters
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BP
Cicatrical Phemigoid Dermatitis Herpetiformis Epidermolysis Bullosa acquisita Eperidmolysis bullosa letalis Herpes gesationis Suction Blister Thermal lesions (burns, cold, LN2) |
"DEET HepSagBC" |
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Basal Lamina and sublaminar connective tissue blisters
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Bullous dermatosis of hemodyalisis
Bulllous eruption SLE Epidermolsis bullosa dystrophica EM (dermal type) ischemic bullae (drug OD) Lichen slcerosus et atrophicus Porphyria cutanea tarda "HIP EEL" |
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Epidermal BM Zone
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Basal K@
anchoring fillaments/Lamina Lucida lamina densa anchoring fibrils interstitial collagen anchoring plaque dermis |
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bullous pemphigoid
pemphigoid gestationis |
anchoring fillaments above lamina densa
attachement to basal K@ |
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Mucous membrane pemphigod
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lower than BP
anchoring fillaments to lamina densa |
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Epidermolysis bullosa acquisita
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anchoring fibrils and interstitial collagen below lamina densa
lamina densa to dermis |
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pemphigus foliaceus autoag
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desmoglein 1
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pemphigus vulgaris
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desmoglein 3 (desmoglein 1)
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paraneoplastic pemphigus
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desmoplakin 1, 2 (desmosomal plaque protein)
desmoglein 3 BP230 envoplakin periplakin |
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anchoring fibrils
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type 7 collagen
lamina densa to anchoring plaques in papillary dermis |
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anchoring plaques
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papillary dermis
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anchroing fillaments
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within the lamina lucida
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BP autoabs
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BP180
BP 230 hemidesmosome and lamina lucida |
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Herpes Gestationis autoabs
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BP180
BP230 hemidesmosome and lamina lucida |
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Cicatricial Pemphigoid autoabs
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BP180
Laminin V hemidesmosome and lamina lucida |
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Linear IgA autoabs
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BP180
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Laminin 5
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anchorning filament-lamina densa of hemidesmosomes
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mucous membrane pemphigoid (cicatricial pemphigoid)
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Lamina 5
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Epidermolysis bullosa acquisita autoag
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Collagen 7
anchoring fibrils |
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IgA pemphigus
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Desmocollin 1
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Indirect Immunfluorescence IgA?
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NO
circulating ab not known indirect immunofluorescence negative |
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Indirect Immunofluorescence how?
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serum clotted patient's blood incubate with animal epi/monkey esophagus
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Dermatitis Herpetiformis
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30s to 40s
extensor knees, buttocks, neck back BURNING, ITCHING SYMMETRICAL PRODROME BURNING THEN LESION THYROID HYPOTHROID 14% HLA-B8 60% HLA class II ags HLA-DR3 95%, HLA-DDQw2 100% celiac-type dental enamel defects (less severe, early childhood had celiac) + TRANSGLUTAMINASE autoabs patchy areas o villous atrophy and inflammation less severe small intestine thatn ordinary gluten senstive enteropathymalabsorption rare < 20% malabsorption fat, D-xylose, or iron IgA antiendomysial abs (IgA-EmA_ severity of gluten induced jejunum damage |
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Linear IgA bullous dermatosis
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LABD
vancomycin 2 o 15 days IgA BMZ +/- IgG NO GLUTEN SENSITIVE ENTEROPATHY +/- circulating IgA anti BMZ abs on indirect IF NO TRANSGLUTAMINASE autoabs |
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IgA-EmA
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severity of villous atrophy
dermatitis herpetiformis no if gluten free diet x one year |
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Dermatitis Herpetiformis cancer?
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Lymphoma
on non-gluten free diet |
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Linear IgA bullous dermatosis
drug? |
LABD
vancomycin 2 to 14 days |
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LABD
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linear IgA at BMZ
no gluten enteropathy some w circulating IgA autoabs No IgA autoabs to transglutaminase (+ in DH) |
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IgA-EmA DH? indicator of ?
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villious atrophy
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Dermatitis Herpetiformis
itch? burn? oral symptoms? |
itch: yes
burn: yes oral symptoms: dryness/ulcers 63% |
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Dermatitis Herpatiforms
IgA anti-tTG abs |
IggA antiendomysial abs to tissue transglutaminase (enzem that metabolized gliadin)
= villious atrophy in jenjunum NOT IN IGA bullous dermatosis |
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Dermatitis Herpetiformis treatment?
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Sulfones
DAPSONE: lysosomal stabilization SULFAPYRIDINE |
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Dapsone side effects?
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dose-related hemolysis, anemia, and methemoglobinemia (BLUE-GRAY CYANOSIS)
Check WBC and CBC q wk x 1 mth then q mth x 6 mths, and then semiannually No G6PD check AA and mediterranean distal upper/lower neuropathy foot drop, hands (rare sensory) agranulocytosis and aplastic anemia (rare) safe pregnancy |
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Medication reduces dose-dependent methemoglobinemia from Dapsone?
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cimetidine
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Sulfapyridine
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medication used DH
no neuropathy no hemolysis short acting sulfonamide sulfasalazine (azulfidine) metabolized to F-ASA (mesalamine) and sulfapyridine |
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Dapsone Hypersensitivity Reaction
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4 weeks after starting
mono-like exanthematous skin hepatitis hypothyroidism 3 months later treatement prednisone |
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enterotoxin in gluten
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gliadin
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DH antibiotics?
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tetracylcline or minocycline with nicotinamide treatment
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Diabetic bullae
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NO PAIN, DEEP
NON-INFLAMMED BASE unknown cause ishemic? |
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Diabetic bullae
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NO PAIN, DEEP
NON-INFLAMMED BASE unknown cause ishemic? |
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paraneoplastic pemphigus cancer?
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lymphoma
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prevalence of pemphigus
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0.1 to 0.5/100,000/yr
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pemphigus HLA?
age of onset? |
HLA-DR4 and HLA-DQ8 (jewish)
HLA-DR6 and HLA DQ5 (non-jewish) 50s |
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Bullous dibeticorum
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no trauma
nonerythematous skin lower legs and feet, but also arms NO PAIN heal spon |
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pemphigus vulgaris oral symptoms?
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> 50% PV with oral lesions (can precede skin by several mths)
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pemphigus vulgaris oral symptoms?
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> 50% oral lesions/erosions
(may precede skin by mths) may involve the larynx |
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Pemphigus etiology
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predisposed and environment?
idiopathic and induced have same HLA |
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Fogo Selvagem
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pemphigus foliaceus in rural S America
ags of infection agent mimic desmoglein 1? = immune response Portuguese for wild ire endemic pemphigus foliaceus Central South america (Brazil, Colombia, Bolivai, Peru, Venezuela, Tunisia) disappears when jungle is cleared KIDS AND YOUNG ADULTS |
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Types of desmoglein
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1, 2, 3
1, 3 stratafied squamous |
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pemphigus vulgaris target molecule
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desmoglein 3 (Dsg3) transmembrane desmosomal component
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mucosal dominant PV target molecule?
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Desmoglein 3 (mucosal)
no desmoglein 1 |
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Pempigus foliaceus target molecule
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desmoglein 1
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Pemphigus Vulgaris
clinical oral lesions? painful? itch? Nikolsky's? scarring? |
MC pempigus
PAINFUL ORAL LESIONS NO ITCH SCALP, FACE, AXILLAE, ORAL CAVITY + Nikolsky's NO scarring (with brown pig) +/- esophageal mucosa mortality 10% complications of steroid tx (previously bc of infection) |
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Pemphigus Vegetans
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variant of PV
large verrucous confluent plaques and pustules FLEXURAL, AXILLAE, GROIN |
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Pemphigus Foliaceus
age of onset? race? oral lesions? distribution? |
wide age compared to PV
no race RARE ORAL LESIONS butteryfly, SCALP, CHEST, UPPER BACK (seb derm) |
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PF
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superficial pemphigus
well demarcated, no large eroded areas desmoglein 1 |
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Pemphigus Erythematous
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PF and SLE
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IgA Pemphigus
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IgA abs epi cell surfaces
similar clinical and histo to subcorneal pustular dermatosis and PF 50% w circulating IgA anti-cell surface abs |
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Pemphigus antibodies DIF
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IgG, strong IgG4, and C3 intercellular
Michel's transport media room temp DIF |
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Inidrect Immunofluorescence PV
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75% pts +
monkey and pig espohagus Dsg1 and Dsg3 no correlate circulating IgG with activity of disease - use clinical |
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Pemphigus treatment
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STEROIDS high dose
topical clobetasol cyclophosphamide axathioprine NO MTX (infections) |
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Cyclophosphamide
side effects? |
pemphigus adjuvant treatment with steroids
BM, hemorrhagic cystitis, bladder fibrosis, reversbile alopecia, increased risk bladder ca, and lymphoma check UA and CBC, drink po fluids |
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azathioprine
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bm, liver, increased mal (loer than cyclophosphamide)
check BC and liver |
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IVIG pemphigus
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can be monotherapy
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pemphigus when to stop treatment?
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DIF bx
80% with neg DIF no pemphigus at 5 yrs |
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Pemphigus assoc with?
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MG abd thyoma (erythematous and vulgaris) (thyoma then MG then Pemphigus)
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Drug induced pemphigus
2 groups |
1. sulfhydryl radical (thiol drugs or SH drugs)
2. non-thiol drugs 10% mortality atuoab same as nondrug pemphigus |
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Sulfhydral/thiol or SH drugs pemphigus
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penicillamine (thiol)
5% patients get pemphigus Captopril thiol drugs spon recovery 40 - 50% (non-thiol only 15% recover) thiol induce pemphigus, non-thiol induce pemphigus in predisposed patients |
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Paraneoplastic pemphigus
clinical? oral lesions? location? other systems involved? cause of death? |
autoimmune
clinical and histo of SJS and PV PAINFUL ORAL ULCERATIONS CONJUNCTIVAL POLYMORPHOUS SKIN LESIONS TRUNK, EXTREMITIES to blisters DESMOSOMES AND HEMIDESMOSOMES respiratory epi BRONCHIOLITIS OBLITERANS does not always coniside with severity of underlying cancer |
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paraneoplastic pemphigus autoabs DIF
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IgG and C3 cells
granular C3 basement membrane histo similar to both em and pv |
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paraneoplastic vs pv?
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use indirect immunoflourescence
rat bladder substrate paraneoplastic circulating IgG anti-cell-sruface and ANTI-CYTOPLASMIC abs |
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BP
age? race? gender? clinical |
elderly
no race no gender drugs |
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BP antigens
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BP230 (cytoplasmic)
BP180 (transmembrane glycoprotein of hemidesmosomes of epi basal cells) IgG circulating and bound to lamina lucida BMZ IgG bound to BMZ activate complement, chemnotzxis, degran of leukocytees proteolytic enzymes mb destruction SUBEPIDERMAL BLISTER |
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Diagnosis of Neoplasia-induced Pemphigus
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3 Major or 2 major and 2 minor
MAJOR 1. Polymorphous mucocutaneous eruption 2. concurrent neoplasia 3. serum immunoprecipitation MINOR 1. + cytoplasmic rat bladder indirect 2. intercellular and bmz on DIF 3. acantholysis |
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BP
oral? preblistering stage? itch? palms and soles? Nikolsky's? spread? heals? compared to pv |
mild oral transient 24%
hives MOD TO SEVERE ITCH PALMS AND SOLES, generalized MC LOWER ABDOMEN, GROIN, FLEXOR EXTREMITIES genitals 7% CLUSTERS Nikolsky's neg no spread, heals rapidly (unlike pv) sites of trauma |
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Variants of PB
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vesicular
vegetating hyperkeratotic erythrodermic |
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generalized BP
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prognosis poor
19% mortality 1 year no fever prog poor BP180 low albumin poor prog |
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BP Labs
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periph eosinophilia 50%
IgE 85% decrease IgE and BP180 IgG when remission |
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BP histo
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subepi blisters
E@ bullae and dermis |
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BP DIF
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IgG +/- C3, +/- IgA,IgM, and fibrin in LINEAR BAND BMZ
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EBA vs BP
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sodium chloride separate lamina lucida
IgG BP on epi side (more IgG w C3) EBA on dermal side |
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azathropine myelosupression?
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TPMT thiopurine methyltransferase activity
intermediate or low |
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BP lesions scarring?
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no scarring no milia
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Cicatricial Pemphigoid
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mucosal
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localized BP? DIF?
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all have circulating IgG
DIF may not be + |
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Localized BP types
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1. Cicatricial (mucosal)
2. localized childhood vulvar pemphigoid 3. pretibial pemphigoid 4. localized chronic pemphigoid (Brunsting-Perry) 5. dyshidrosiform pemphigoid 6. pemphigoid vegetans same circulating IgG as BP DIF may be neg |
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Pretibial Pemphigoid
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localized BP shins young women
nonscarring |
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Brunsting-Perry
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Localized chronic pemphigoid
crops of grouped blisers on head and neck heal w atrophic scars |
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dyshidrosiform pemphigoid
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Localized BP
vesiculobullous hemorrhagic lesiosn palms and soles |
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pemphigoid vegetans
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Localized BP
erosive and vegetating plaques |
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Cicatricial Phemigoid
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Mucousal Membrane Pemphigoid (MMP)
rare chronic subepi blistering SCARRING lamina lucida 40+ yrs 2F:1M ORAL CAVITY AND EYE FEW REMISSIONS (unlike BP) |
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MMP
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Cicatricial Pemphigoid
oral 85% DESQUAMATIVE GINGIVITIS (mc) PAINLESS nonilamed erosions NO VERMILION BORDER (unlike pemphigus) HOARSENESS 8% (laryngeal) only oral - better prognosis |
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MMP
eyes? |
mucosal membrane pemphigoid
ocular disease 65% UNILATERAL conjunciviis then BILATERAL FIBROSIS CONJUNCIVIAL EPI shrinkage of conjunctival obliterateion of conjunctival sac CORNEAL OPFACIFICAION AND PERFORATION BLINDNESS 20% remission 22% relapse |
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MMP treatment
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topical steroids, intralesional steroids
DAPSONE then prednisone w/o dapsone Cyclophosphamide then azatioprine |
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MMP eyes topical steroids?
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no effective
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Benign chronic bullous dermatosis of childhood
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NONHEREDITARY
ONLY MODERATE ITCHING CLUSTERS ON FACE (around mouth), LOWER TRUNK, INNER THIGHS, GENITALS LINEAR IgA BMZ, circulating IgA abs NORMAL JEJUNAL BX remissions and relapses then no after puberty DAPSONE then steroids |
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Herpes Gestationis (HG)
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Pephigoid Gestationis
VERY ITCHY 1/50,000 preg class II ags:HLA-DR3, HLA-DR4 class III ag: C4 IgG to 180 of hemidemosomes 2nd or 3rd trimester 2nd week postpartum 10% newborn cutaneous increase prematurity |
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Herpes Gestationis
location |
crops on abdomen and exremities and coalesce polycylic rings
NO SCARRING post inflam hyperpig menses and OCPs |
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HG DIF
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BANDLIKE C3
IgG 10% circulating IgG Herpes Gestationis Factor fixes complement use complement innumolfluorescence placenta transietn pemphigoid like skin lesions PERIPH EOSINOPHILIA |
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HG Factor
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fixes complement
use complemtn immunofluorescence placenta no indirect immunoflourescence |
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HG skin lesions?
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polycyctic rings
abdomen and extremities |
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EBA
loc of blister? scarring or milia? response to therapy? age? |
Epidermolysis bullosa acquisia
rare chronic subepi mucocuaneous trauma SCARRING AND MILIA poor response to therapy kids and adults |
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EBA autoab to?
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type 7 collagen anchoring fibrils
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Classic EBA
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trauma blisters
MILD MUCOUS MEMBRANE SCARS BLISTERS ON NON-INFLAMMATORY BASE PALMS, SOLES, KNEES scarring alopecia and nail dystrophy OCULAR COMMON blindness rare |
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BP-like BBA
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50% EBA early disease
TENSE BLISTERS ERYTHEMATOUS INFLAMMATORY BASE TRUNK AND FLEXURAL ICHING some lesions with scarring and milia |
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EBA DIF
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LINEAR and HOMOGENOUS IgG and C3 MZ
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Hailey-Hailey
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rare auto dom
exposed to UV light LONGITUDINAL WHITE BANDS fingernail 71% SUCTION on normal skin subclinical abnormality k@adhesion UV provcation test |
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Hailey-Hailey
nonintertriginous lesions |
group pruritic vesicles red or noniflamed base
grouped in ANULAR OR SERPIGINOUS pattern rupture quickly and ADVANCING RIM OF SCALE and crust pale hhypogi center crops of vesicles on border heal spon in colder weather |
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Hailey-Hailey Intertriginous Lesions
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mosit red fissured areas or vegetating warty papules and palques do not extend beyond opposing skin surfaces of the groin or aillae
chronic |
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Hailey-Hailey treatment
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nonintertriinous lesions: oral ab, topical steroids
Intertriginous Lesions: topical steroids |
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Epidermolysis bullosa
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3 groups
16 variants genetic minor trauma NON-INFLAMMATORY blisers MECAHNOBULLOUS DISEASES SCARRING OR NON-SCARRING Epidermolysis bullosa simplex 92% Dystrophic epidermolysis bullosa 5% Jxnal epidermolysis bullosa 1% |
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EB clinical classificaion
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scarring vs nonscarring
epidermolysis bullosa simplex no scarring jxnal epidermolysis bullosa atrophy dystrophic epidermolysis bullosa scarring |
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Histo EB classification
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EBS epi basal layer
JEB BMZ DEB dermis |
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Epidermolysis Bullosa Simplex
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AUTO DOM
SPORADIC INFANCY OR CHILDHOOD HAND AND FEET or truma NO SCARRING INFECTION |
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Jxnal Epidermolysis Bullosa
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AUTO RECESSIVE
NO PALMS AND SOLES INFANCY ATROPHY NO SCARRING MOUTH, LARYNX, EYES, ESOPHAGUS DIE CHILDHOOD |
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Dystrophic Epidermolysis Bullosa
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AUTO DOM OR AUTO RECESSIVE
SEVERE RECESSIVE cycles of blistering and SCARRING MITTEN DEFROMITY |
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EB dx
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EM
Monoclonal abs |
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EB treatment
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avoid trauma
DILANTIN (collagenase inhibitor) not useful for recessive DEB |
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Weber-Cockayne Disease
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EBS mild blistering hands and feet
AUTO DOM |
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Newborn w blisters, pustules, erosions, and ulcerations
tests? |
bacterial, viral, fungal, gram stain, wrigh's stain, tzanck smearl, KOH, and skin bx
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