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Cutaneous Autoimmune Blistering Disease: 

Cutaneous Autoimmune Blistering Disease ~ Pathology Case Review ~2004, March/Aprial

Cutaneous blister: 

Cutaneous blister Result of significant spongiosis, balloning degeneration, basal vacuolopathy, inherited disorder or autoimmune destruction Histology, DIF and clinical history to arrive at the correct diagnosis

Case 1: 

Case 1 24 y/o black woman with tense 2-3 cm fluid-filled blister on the upper extremity, joint pain and swelling Hx of SLE progress to involve the forehead and lower extremity LM: subepidermal blister with a dense neutrophilic infiltrate

Case 1: 

Case 1 DIF: linear Ig G, IgA and IgM deposition along the BM IIF: deposition along the floor of the skin

Case 1: 

Case 1 Beyond the mechanism of disruption, the levels of separation and the type of inflammatory responses help to further characterize the process Confirmation of autoantibody deposition: DIF, IIF, immunoblot, immunoprecepitation Immnoelerctorn microscopy Salt split skin : subepidermal blistering dx

Porphyria cutanea tarda(PCT): 

Porphyria cutanea tarda(PCT) Sun exposed sites Paucicellular subepidermal blister Reduptication of the vascular and epidermal basement membrane DIF: IgG and C3 trapped in vascular wall

Epidermolysis bullosa acquisita (EBA): 

Epidermolysis bullosa acquisita (EBA) Blister on acral and trauma-prone sites DIF: IgG and C3 deposition along the floor of separation Other D/D: urticarial variant of BP, suction blister, blister due to cryotherapy or heat, inherited epidermolysis bullosa variant, TEN

Bullous pemphigoid(BP): 

Bullous pemphigoid(BP) Over 60 y/o, bullae on the trunk and extremity Subepidermal blister with inflammatory infiltrate( predominant eosinophil) DIF: linear IgG along the BM IIF: deposition on the roof

Dermatitis herpetiformis(DH): 

Dermatitis herpetiformis(DH) Adult with a chronic pruritic eruption Strong genetic predisposition DIF: IgA and C3 in the tip of dermal papilla

Linear IgA dermatosis: 

Linear IgA dermatosis Childhood and adult type DIF: not just localized to the dermal papilla but also along the dermal epidermal junction

Cicatrical pemphigoid(CP): 

Cicatrical pemphigoid(CP) Oral and conjuctival blister in elderly p’t autoAb: direct against BPAg2 of the hemidesmosome, dpiligrin in the lamina densa and type VII collagen

Bullous systemic lupus erythematosus(BSLE): 

Bullous systemic lupus erythematosus(BSLE) An uncommon manifestation of SLE PMN deeper in the papillary dermis and around vessel DIF: linear or granular deposition of IgG and complement along the BM Direct against collagen VII

Case (2): 

Case (2) 38 y/o man with extensive painful erosions of the lip and tongue Polymopphous lichenoid plaque, LAPs LM: basal vaculopathy with a suprabasal blister, lymphocyte exocytosis, keratinocyte necrosis, suprabasal acantholysis DIF: BM, intercellar deposistion IgG

Pemphigus foliaceous(PF): 

Pemphigus foliaceous(PF) Subcorneal bilster on the head and neck Never involve mucosal surface Ab to desmoglein DIF: IgG fishnet pattern

Pemphigus erythematosus: 

Pemphigus erythematosus Well-circumscribed lesions on the malar area DIF: IgG,C3 in both BM and intercelluar 30% positive ANA and associated with other autoimmune disease

Pemphigus vulgaris(PV): 

Pemphigus vulgaris(PV) Painful oral ulcer Blister at the most basal layer of the epidermis, loss of cell-cell adhesion with maintenance of attachment to the BM DIF: IgG, C3 on the keratinocyte, Ab to desmoglein

Paraneoplastic pemphigus(PNP): 

Paraneoplastic pemphigus(PNP) Occurs exclusively in the neoplasm Complex set of autoAb to one or more proteins that play key roles in cell-cell adhesion Significant variability in histologic features IIF:intercelluar staining

Other D/D: 

Other D/D Acute spongiosis, viral infection, Hailey-Hailey disease, Darier disease, Grover disease, friction blister

Summary: 

Summary Cutaneous autoimmune bilstering dx have many overlapping clinical and histological features and rely on clinicopathological correlation The level or separation, the type of inflammatory infiltrate and IF pattern nonAb mediated mediated process

Our experience: 

Our experience

Slide32: 

Other: epidermolysis bullosa simplex EBA EM Lichen plans pemphgioids Bullousis diabeticum H.P. purpura Bullous amyloidosis Granuloma faciala Pigmented purpura dermatosis

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