Langerhans' cell histiocytosis (histiocytosis-X) in a women with typical ultrastructure of Birbeck's granule: a case report.

A 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.