Bullous pemphigoid

Synonym(s)

Pemphigoid; pemphigoid vegetans Definition

Autoimmune, blistering disease characterized by the presence of IgG autoantibodies specific for the hemi-desmosomal antigens

Bullous pemphigoid. Numerous tense vesicles and bullae, many of which arise from normal-appearing skin

108 Bullous photosensitivity, drug- or therapy-induced

Pathogenesis

IgG autoantibodies specific for the hemi-desmosomal bullous pemphigoid antigens BP230 (BPAg1) and BP180 (BPAg2); binding of antibodies at the basement membrane, activating complement and inflammatory mediators and producing injury at the basement membrane zone

Clinical manifestation

Tense vesicles and bullae, with a predilection for the flexor areas of the skin; oral and ocular mucosa involvement seldom occurs; bullae clinically either inflammatory or non-inflammatory; blisters usually heal without scarring or milia formation; localized form with blisters confined to the extremities; lesions sometimes urticarial without vesiculation

Differential diagnosis

Cicatricial pemphigoid; herpes gestationis; linear IgA bullous dermatosis; dermatitis herpetiformis; chronic bullous dermatosis of childhood; dyshidrosis; bullous lupus erythematosus; pemphigus vegetans; urticaria

Therapy

Mild to moderate disease: high potency topical corticosteroids; combination of tet-racycline and niacinamide 500 mg PO 2-3 times daily

Severe disease: prednisone; steroid-sparing medications: azathioprine; cyclophospha-mide; mycophenolate mofetil; dapsone; methotrexate

References

Khumalo NP, Murrell DF, Wojnarowska F, Kirt-schig G (2002) A systematic review of treatments for bullous pemphigoid. Archives of Dermatology 138(3^385-389

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