Cicatricial pemphigoid (CP) is a rare blistering disease characterized by erosive lesions of the mucous membranes and skin that result in scarring. The mean age of onset is in the early 60′s and there is a slight female predominance. There is no known racial or geographic clustering. Other names for this disorder include benign mucous membrane pemphigoid, desquamative gingivitis, and ocular pemphigus.
Clinically, oral and conjunctival mucous membranes are most frequently affected and are typically the first sites of involvement. In the mouth, the gingiva, buccal mucosa, and palate are typically involved and patients may present with mucosal erosions or tense blisters. Adhesions may develop in severe disease and gingival involvement can result in loss of teeth. Ocular involvement typically manifests as unilateral or bilateral conjunctivitis or as burning, dryness, or foreign body sensation. Severe disease can lead to scarring and vision loss. Other mucosal sites that may be affected include the nasopharyngeal, laryngeal, esophageal, genital, and rectal mucosa. Cutaneous lesions are present in about one third of patients. The head, neck and upper trunk are most frequently involved. Typically, patients have a few scattered erosions or tense blisters on a red or urticarial base. However, the extent and number of cutaneous lesions is usually small.
On electron microscopic exam, blister formation is in the lamina lucida. On histopathologic exam, lesional skin shows subepidermal bullae with a mixed leukocytic infiltrate. Mononuclear cells, histiocytes, and plasma cells predominate in mucosal lesions, while eosinophils and neutrophils are commonly seen in skin lesions. Direct IF of perilesional tissue demonstrates linear IgG and C3 at the basement membrane zone most commonly. Mucous membranes are the preferred biopsy site for direct immunofluorescence. Indirect immunofluorescence is usually negative. Autoantibodies from patients with cicatricial pemphigoid may recognize a variety of different antigens present in epithelial basement membranes. Most patients with circulating IgG that binds to the epidermal side of salt split skin have autoantibodies that react with BPAG 2. However, some patients have IgG directed against laminin 5 (epiligrin) which localizes to the dermal side of salt split skin.
This disease is chronic and progressive. Spontaneous remission is rare.