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Chronic nonhereditary blistering disease of the skin or mucosae. Usually considered an autoimmune disease.

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Bullous Pemphigoid Brunsting-Perry type.

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Bullous pemphigoid affects the skin; cicatricial pemphigoid affects the mucosal surfaces of the body and, occasionally, the skin.

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Clinical appearance of the eruption and skin biopsy.

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Bullous pemphigoid: Multiple tense bullae arising from large, irregular urticarial plaques; lesions occur most typically on the flexion surfaces of the extremities or lower torso but may be generalized in severe cases. It may occur at any age, but is most common in the elderly. Clinical course of exacerbations and remissions.

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Cicatricial pemphigoid: Bullous eruptions involving mainly the oropharynx, conjunctiva, larynx, esophagus, genitalia, and anus. The scarring of the mucosal surface as a consequence of the recurrent blistering can result in blindness, nasal or laryngeal obstruction, or esophageal stenosis.

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It is often associated with dermatitis herpetiformis, also with multiple sclerosis (with no involvement of mucous membrane, but with an early onset). It is described as associated with autoimmune thrombocytopenia, nephropathy, and ulcerative colitis.

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History of the associated disease: renal, ulcerative colitis, multiple sclerosis. History of medications, usually high-dose corticosteroids, dapsone, azathioprine, and/or other immunosuppressive agents such as cyclosporin.

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The same considerations as for the other blistering diseases: difficult venous access, friction of the skin or mucosae may cause blistering; careful padding; avoid tapes; use petroleum jelly gauze; lubricate blade and tubes well. In case of cicatricial pemphigoid, airway instrumentation should be avoided if possible.

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Many medications can induce pemphigoid; the more common are furosemide, ibuprofen, d-penicillamine, α-aminobenzylpenicillin, penicillin G, sulfasalazine, 5-fluorouracil, 8-methoxypsoralen; it is best to avoid these medications if possible.

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Chronic Benign Bullous Dermatosis of Childhood: Large tense bullae of the skin, resembling bullous pemphigoid, occurring in preschool-age children. Spontaneous remission usually occurs within 2 or 3 years. Therapy: corticosteroids, sulfa drugs.

Fellner MJ: Drug-induced bullous pemphigoid. Clin Dermatol 11:515, 1993.  [PubMed: 8124641]

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