Cicatricial pemphigoid.
This case of CP is of interest because of its "not-so-benign" course in this patient, its unusual immunofluorescence patterns, and the need for a complex therapeutic regimen to achieve control. This patient had severe ocular; laryngeal, and oropharyngeal involvement leading to visual problems, hoarseness, and marked weight loss and dehydration. He also had anemia thought to be partially related to dapsone use. We believe the side effects of dapsone, combined with fluid retention due to prednisone therapy, contributed to cardiac failure. The diagnosis of CP is usually established by correlation of clinical findings with immunofluorescence studies. However, indirect immunofluorescence may show strong intercellular antibody binding in the epidermis (ie, pemphigus-like antibodies). Treatment alternatives for patients with CP who have adverse reactions to, or no significant benefit from, conventional agents such as dapsone or prednisone may include immunosuppressive agents such as azathioprine or cyclophosphamide. As this case demonstrates, care of patients having CP involves a cooperative effort from a number of different specialties, including dermatology, primary care, ophthalmology, and otolaryngology.
Warren
S D
SD
Department of Dermatology, Medical College of Georgia, Augusta 30912-2900.
Lesher
J L
JL
D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use*; D003622:Dapsone / Q000627:therapeutic use; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use
D003622:Dapsone; D001379:Azathioprine; D011241:Prednisone