- Bullous pemphigoid is an autoimmune blistering disease that presents in elderly patients as urticarial papules and plaques and tense bullae often accompanied by pruritis. It is extremely rare in children. It is characterized by autoantibodies against hemidesmosome proteins of the basement membrane.
- The diagnosis of bullous pemphigoid is generally made based on clinical findings supplemented by histologic evaluation and immunofluorescence. Histologic evaluation usually reveals subepidermal blister formation. Biopsy of perilesional skin for direct immunofluorescence will usually identify a linear band of IgG and C3 antibodies along the dermo-epidermal junction.
- Bullous pemphigoid often requires systemic immunosuppression to adequately control the disease, usually with corticosteroids followed by a steroid-sparing agent; chronic, long-term treatment is generally required.