Images in Clinical Medicine
Cicatricial Pemphigoid
N Engl J Med 2004; 350:2499June 10, 2004
- Article
A 26-year-old man presented with blisters and erosions that had developed on his tongue over a 48-hour period (Panel A). The blisters had appeared primarily in his mouth, but several had also appeared on his face and trunk. There was no involvement of his eyes. The major complication has been laryngeal scarring due to blister formation.
Two years before this presentation, a biopsy showed an infiltrate of eosinophils in a subepidermal blister. Direct immunofluorescence of the biopsy specimen showed a linear deposition of IgG on the basement membrane. These findings were consistent with a diagnosis of cicatricial pemphigoid. On indirect immunofluorescence, circulating anti-skin basement-membrane–zone antibodies of the IgG and IgA classes were also detected, at titers of 1:20 and 1:80, respectively. The patient required emergency surgery with a carbon dioxide laser on two occasions to relieve acute stridor caused by laryngeal obstruction. He has been treated with dapsone, tetracycline, and niacinamide. While receiving treatment with 200 mg of azathioprine per day and 10 mg of prednisone per day, the patient has had no recurrence of blistering for the past 18 months.
A second patient, a 70-year-old man, had had cicatricial pemphigoid confined to his right eye for three years. A biopsy of his conjunctiva showed IgA and IgG antibodies deposited in the basement-membrane zone. Despite treatment with high-dose prednisone and cyclophosphamide, vision in the affected eye progressively deteriorated because of corneal scarring due to symblephara (Panel B, arrows) and trichiasis (arrowhead). Treatment with 250 mg of azathioprine per day for the past two years has halted the deterioration of his vision.
Conleth A. Egan, M.R.C.P.I.
Scott R. Florell, M.D.
University of Utah Health Sciences Center, Salt Lake City, UT 84132
