Pemphigus

Abstract
SELECTED CASES Case 1.— A 47-year-old woman presented to the National Institutes of Health Clinical Center for evaluation of a 3-year history of painful oral erosions (Fig 1, top left). During the previous 2 months her oral erosions were so extensive and severe that she could not eat well and lost approximately 9 kg. In the last month she also noted erosions on the skin of her trunk and extremities. A skin biopsy specimen revealed an intraepidermal blister with acantholysis (loss of cell-to-cell adhesion) just above the basal cell layer (Fig 1, top right). The results of direct immunofluorescence of perilesional skin were positive for IgG on the cell surface of keratinocytes throughout the epidermis. Indirect immunofluorescence showed a serum IgG that bound to the epithelial cell surface of monkey esophagus at a titer of 160. Pemphigus vulgaris (PV) was diagnosed, and the patient was treated daily with 80 mg