Cicatricial pemphigoid in the upper aerodigestive tract: diagnosis and management in severe laryngeal stenosis.
Pemphigoid is a group of rare, acquired, autoimmune subepithelial blistering diseases. The condition has been subclassified into bullous pemphigoid and cicatricial pemphigoid (CP). Diagnosis is based on clinical presentation, evidence of subepithelialvesicles or bullae on routine histologic analysis, and direct and indirect immunofluorescence studies. Cicatricial pemphigoid is characterized by linear deposition of immunoreactants, principally IgG and complement factor 3, along epithelial basement membranes. Cicatricial pemphigoid usually leads to mucosal scarring. We present a case of severe CP that led to laryngeal and subglottic stenosis and involvement of both eyes and the oral, nasal, and nasopharyngeal mucosae. Treatment with dapsone, corticosteroids, azathioprine sodium, cyclosporine A, cyclophosphamide, methotrexate sodium, and mycophenolate mofetil between 1997 and 2001 only resulted in temporary disease control. The patient has been treated with leflunomide for the past 8 months, and there have been no relapses. Treatment of CP with leflunomide has not been described in the literature until now.
Boedeker
Carsten Christof
CC
Department of Otorhinolaryngology-Head and Neck Surgery, Albert-Ludwigs-University, Freiburg, Germany.
Termeer
Christian Carl
CC
Staats
Richard
R
Ridder
Gerd Jürgen
GJ
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007555:Isoxazoles / Q000627:therapeutic use*; D007829:Laryngostenosis / Q000209:etiology*; D000077339:Leflunomide; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D007555:Isoxazoles; D011239:Prednisolone; D000077339:Leflunomide