Oral pemphigoid: Subset of cicatricial pemphigoid?

• Published in: Oral surgery, oral medicine, oral pathology, oral radiology and endodontics Vol. 85; no. 1; pp. 37 - 43
• Main Authors: Mobini, Narciss, Nagarwalla, Neville, Ahmed, A.Razzaque
• Format: Journal Article
• Language: English
• Published: St. Louis, MO Mosby, Inc 1998; Elsevier
• Subjects: Adult; Aged; Aged, 80 and over; Antibodies; Basement Membrane - immunology; Biological and medical sciences; Dapsone - therapeutic use; Dentistry; Female; Follow-Up Studies; Gingival Hemorrhage - etiology; Gingivitis - etiology; Humans; Male; Medical sciences; Middle Aged; Mouth Diseases - drug therapy; Mouth Diseases - etiology; Mouth Diseases - pathology; Mouth Mucosa - pathology; Mouthwashes - therapeutic use; Non tumoral diseases; Otorhinolaryngology. Stomatology; Pemphigoid, Bullous - complications; Pemphigoid, Bullous - drug therapy; Pemphigoid, Bullous - pathology; Remission Induction; Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology; Bullous dermatosis; Human; Skin disease; Prognosis; Scarring pemphigoid; Sulfone; Stomatology; Dapsone; Clinical form; Autoimmune disease; Review; Oral cavity; Symptomatology; Chemotherapy; Treatment; Oral cavity disease; Localization
• ISSN: 1079-2104; 1528-395X
• DOI: 10.1016/S1079-2104(98)90395-X

Objective. Cicatricial pemphigoid (CP) is an autoimmune blistering disease characterized by anti-basement membrane zone (BMZ) antibodies with a varied heterogeneous clinical spectrum. We sought to characterize a subset of patients with disease limited to the oral cavity. Study Design. Twenty-nine random patients with vesiculobullous disease limited to the oral cavity were studied. We identified patients by clinical criteria, the presence of subepidermal/subepithelial bullae on routine histopathologic study, and deposition of IgG, complement, or both on the BMZ of perilesional tissue by immunopathological studies. Treatment included local and systemic therapies. Patients were monitored for a mean period of 6.7 years (range, 3.5 to 11 years). Results. The female/male ratio was 4.8:1. Patients with limited or minimal disease received local therapy only. In patients with extensive or severe disease, the use of dapsone yielded significant clinical improvement. Long-term follow-up showed that patients with severe disease treated with dapsone followed a clinical course similar to that in patients with minimal disease. Conclusions. Oral pemphigoid is a distinct clinical subset of CP. Overall it has a relatively benign course compared with that in patients with CP involving the oral cavity and other mucosae and the skin. Patients with minimal disease respond satisfactorily to topical therapy. Patients with severe and extensive disease benefit from dapsone therapy. In most patients the clinical course is prolonged and treatment is required for several months; in our study the mean treatment period was 42 months (range, 24 to 78 months). All the patients in this study went into clinical remission and remained in remission on cessation of therapy. No other mucosae or the skin were involved during the follow-up period.