A CASE OF CONGENITAL SELF-HEALING HISTIOCYTOSIS INVOLVED IN THE SKIN,ORAL-MUCOSA AND VISCERAL ORGANS AND A REVIEW OF JAPANESE LITERATURE

A male infant had numerous hemorrhagic vesicles of 3 to 10 mm in size over the entire skin at birth. A solitary yellowish nodule in the oral mucosa and a dark red tumor in the right palpebral conjunctiva were a lso noted. Skin biopsy confirmed the diagnosis of Langerhans cell hist iocytosis. On day 19, the patient developed lesions in the lungs and t hymus without respiratory symptoms. Without any therapy, all cutaneous , mucosal and visceral lesions spontaneously regressed by 5 months of age. Since then, the patient has had no recurrence for 30 months and i s doing well, indicating that this patient had congenital self-healing histiocytosis. We reviewed the clinicopathologic features of 14 cases of congenital self-healing histiocytosis documented in Japan; however , except for this patient, none showed mucous and visceral lesions.