Pemphigus vulgaris. Report of a case with electron microscopic observations of the lesion.

Pemphigus vulgaris is a chronic autoimmune bullous disease with a severe prognosis which involves the epidermis and mucous membranes.A 24-year-old woman was referred to our clinic for diagnosis and treatment of bullae and erosion of oral mucosa.Intraoral examination revealed numerous extensive erytheraatous erosion on the palate, tongue, buccal mucosa, and labial mucosa.Specimen for biopsy was obtained from lower lip mucosa.Histopathological features showed suprabasal intraepithelial bulla formation with acantholysis typical of pemphigus vulgaris.Direct immunofluorescent staining demonstrated intercellular substance deposition of IgG.Ultrastructural investigation in oral pemphigus vulgaris showed loss of desmosomes, widened intercellular spaces and the tonofilaments accumulated around the nucleus.The diagnosis of pemphigus vulgaris was established, and treatment with 40mg prednisone daily was instituted. With progressive improvement the dosage was tapered.The skin and oral lesions remitted and a maintenance dose of 5mg of prednisone every other day and 25mg of gold sodium thiomalate once a week was instituted.