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Pemphigus, a disease of adults, is characterized by the development of bullae, or blisters, on normal skin. The bullae resolve, leaving pigmented or colored spots. Brunsting-Perry pemphigoid is characterized by areas of recurrent blistering on the head and neck, which result in reddened patches and atrophic scars, or wasting away of skin tissue. The skin lesions of this disease are similar to those of other skin disorders, including basal cell carcinoma, a malignancy of the skin characterized by white-bordered, bleeding ulcers. Three cases are described of patients with skin lesions similar to those found in multicentric basal cell carcinoma. Histologic, or microscopic, tissue examination of the lesions did not provide any specific findings. However, an immunological technique called immunofluorescence indicated changes in various immune factors, substances involved in the body's natural defense mechanisms, which are characteristic of Brunsting-Perry pemphigoid. In cases with minimal blistering, the use of immunofluorescence provides a more accurate diagnosis of Brunsting-Perry pemphigoid. (Consumer Summary produced by Reliance Medical Information, Inc.)

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.)

Herpes simplex, a viral infection usually affecting the skin or nervous system, has rarely been associated with pemphigus vulgaris, an uncommon disease characterized by large blisters of mucocutaneous surfaces, such as inside the mouth. Pemphigus vulgaris is often fatal if left untreated, but may respond to corticosteroid treatment. This is the eighth reported case of herpes simplex and pemphigus vulgaris and occurred in a 55-year-old man with an eight-week history of ulcers in the mouth, fever and tender cervical (neck) lymph nodes. Laboratory cultures for bacteria and fungus were negative; direct viral immunofluorescence was positive for herpes simplex type 1, which was confirmed by viral culture. The patient was treated with acyclovir, but did not improve. A biopsy taken from the mouth provided a diagnosis of pemphigus vulgaris with concurrent herpes simplex of the skin and mouth. The patient was then prescribed prednisone and azathioprine, and initially continued on acyclovir. His condition was controlled by a maximum dose of 340 mg/day of prednisone which was gradually decreased over a five month period to 15 mg/day. In the seven previously reported cases of herpes simplex and pemphigus vulgaris laboratory testing confirmed the diagnosis. Pemphigus vulgaris preceded the herpes infection in five of the seven cases. All of the patients were treated with steroids. There are two additional reported cases of pemphigus vulgaris associated with possible herpetic infection, but a diagnosis of herpes was not definitely proven. (Consumer Summary produced by Reliance Medical Information, Inc.)