Abstract
We report a case of congenital Langerhans cell histiocytosis with skin and digestive tract involvement that resulted in severe growth retardation. A congenital herpetic infection was initially suspected because papulovesicles were intermixed. However, the skin eruptions persisted and later severe growth retardation developed as a result of prolonged diarrhea containing blood and mucus, and hypoproteinemia and edema caused by protein-losing enteropathy. The skin and colon biopsy established the diagnosis of LCH, and no infiltration to other organs was found. Administrations of vinblastine and prednisone with central venous alimentation were very effective. Congenital self-healing LCH (CSHLCH) with spontaneously involuting skin lesions and generally good prognosis has been known as a unique entity of rare congenital LCH. A computer search revealed several case reports very similar to ours, and thus it is suggested that a subtype of congenital LCH that is characterized by skin and digestive tract infiltration and is different from CSHLCH may exist.
