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The bullae are formed by an immune reaction, initiated by the formation of IgG [citation needed] autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, [6] and/or type XVII collagen, also called bullous pemphigoid antigen 2, [7] which is a component of hemidesmosomes. A different form of dystonin is associated with ...
Disease Reason not believed to be autoimmune Cit. Agammaglobulinemia: An immune system disorder but not an autoimmune disease. Amyloidosis: No consistent evidence of association with autoimmunity. Amyotrophic lateral sclerosis: No consistent evidence of association with autoimmunity. Anti-tubular basement membrane nephritis
Bullous pemphigoid is primarily a disease of older adults and it rarely occurs in children. The vast majority of cases involved individuals between the ages of 60 and 80 years. Two European studies have also suggested the increased risk of bullous pemphigoid with advancing age. [28] [29]
It is absent in bullous pemphigoid, making it useful for differential diagnosis. This histological feature is also seen in herpes simplex infections (HSV 1 and 2) and varicella zoster infections (chicken pox and shingles).
Adult linear IgA disease; Bullous pemphigoid; Bullous lupus erythematosus; Childhood linear IgA disease (chronic bullous disease of childhood) Cicatricial pemphigoid (benign mucosal pemphigoid, benign mucous membrane pemphigoid, ocular pemphigus, scarring pemphigoid) Dermatitis herpetiformis (Duhring disease)
Adult linear IgA disease is an acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance. [2] This disease can often be difficult to treat even with usually effective medications such as ...
Antibody isotype(s) and location of antibody deposition in immunofluorescence studies using salt-split skin for autoimmune bullous conditions targeting the basement membrane zone of the human integumentary system Condition Antibody isotype(s) deposited Localization of antibody with use of salt-split skin Antiepilegrin cicatricial pemphigoid ...
[6] [7] It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin epidermal-epidermal junctions ...