Dermatopathology I

Disease	Pathogenesis	Pathology	Clinical Features/ Treatment
Pemphigus Vulgaris	antibodies to keratinocytes, autoimmune IgG to desmoglein 3	seperation of stratum spinosum and outer epidermal layers from basal layer blister - moderate lymphocytes, macrophages, eosinophils, neutrophils	blistering skin large, scalp, mucous membranes, periumbilical and intertriginous areas
Diseases of the Basement Membrane Zone
Epidermolysis Bullosa	hereditary	splitting of epidermis at or near basement membrane	blisters
Epidermolytic EB	disruption of basal keratinocytes	vacuoles filled with abnormal keratin 5 and 14	blisters from minor trauma - no scarring
Junctional EB	AR benign - mutation in gene encoding for type XVII collagen severe- laminin and integrin genes	lamina lucida blister roof - intact epidermis base - lamina densa	blisters
Dermolytic EB	defect in anchoring fibrils, defective collagen type VII	roof - normal epidermis, lamina lucida, lamina densa floor - outer part of papillary dermis	atrophic scarring
Bullous Pemphigoid	complement fixing IgG antibodies to BPAG1 and BPAG2	subepidermal blisters roof - intact epidermis base - lamina densa cytology - numerous eosinophils, arranged along BMZ seperation at lamina lucida	large, tense blisters erythematous base +/- T - corticosteroids

Source: Rubin's Pathology 4th Edition