The pemphigoid spectrum

Bullous pemphigoid (BP) is a subepidermal autoimmune blistering skin disease, and is predominantly a disease of the elderly in its classic presentation. There are distinct clinical variants of this disease: bullous pemphigoid, cicatricial pemphigoid, and pemphigoid gestationis. The autoimmune response in BP is directed against two hemidesmosomal antigens, BP180 (BPantigen2 or collagen XVII) and BP230 (BPantigen1). In CP, autoantibodies recognize multiple auto antigens, including BP180, BP230, laminin-5, laminin-6, and integrin B4 subunit. Autoantibodies in pemphigoid gestations are maily reative with BP180. The diagnosis of this condition is based on clinical findings, histology, and the demonstration of tissue bound immunoreactants (direct immunofluorescence). Therapy can range from topical glucocorticosteroids for mild disease, to oral antibiotics, to systemic glucocorticosteroids alone or with cytotoxic drugs or intravenous immunoglobulin (IVIG).