Pemphigus vulgaris of the esophagus in women

Pemphigus vulgaris, a rare disease characterized by large blisters of mucocutaneous surfaces, is often fatal if left untreated. This disease is mediated by Immunoglobulin G antibodies. A 40-year-old Puerto Rican woman developed painful erosions of the mouth and tongue, and blisters on the trunk and extremities over a six week period. A skin biopsy diagnosed pemphigus vulgaris. Immunofluorescent study revealed heavy deposits of Immunoglobulin G in the epidermis of the skin. The patient was treated with prednisone, but when followed-up four weeks later she had difficulty swallowing and chest pain. She was admitted to the hospital and endoscopy revealed severe esophagitis with exudate (inflammation of the esophagus with pus). The prednisone dose was increased, and the patient quickly improved. Nine other cases of pemphigus vulgaris are reported. The patients were middle-aged women and none had skin lesions when the disease of the esophagus was diagnosed. Five were considered to be improved with neither skin nor mucosal lesions, when the esophagus became affected by the disease. Fiberoptic endoscopy, which uses a flexible tube with a magnifying lens and light to visually examine the esophagus and stomach, appears to be a safe procedure in these patients. It is recommended that endoscopy be performed on any patient with pemphigus vulgaris or a history of this disease who has esophageal symptoms and does not respond appropriately to corticosteroid or other therapy. (Consumer Summary produced by Reliance Medical Information, Inc.)