Pemphigus of the Air and Food Passages

Abstract

Pemphigus vulgaris is one among many bullous diseases which involve the mucous membranes of the oropharynx and the larynx. The pernicious nature and previous high mortality of this disease have been greatly reduced with early diagnosis and the appropriate use of corticosteroids and immunosuppressive agents. Thirteen patients with pemphigus vulgaris were seen at Rush-Presbyterian-St. Luke's Medical Center from 1970 through 1976. All patients had moderate-to-severe generalized eruptions and were biopsy-positive for pemphigus vulgaris. A moderate prednisone dosage of 80 to 120 mg/day in moderate-to-severe cases was utilized in 11 out of 13 patients. All patients were treated initially with prednisone only, and after control of the acute generalized eruptions was achieved, Cytoxan® was added to the regimen to allow reduction of the prednisone dosage. One patient in our series died as a result of disseminated herpes simplex, probably secondary to high-dose corticosteroid treatment. Mortality in our series was 7.6% This investigation suggests that lower prednisone doses of 80 to 120 mg/day, except in recalcitrant cases, may be efficacious in the treatment of pemphigus vulgaris, especially in conjunction with adjuvant immunosuppressive therapy.