We report a case of congenital Langerhans cell histiocytosis (LCH), presenting with a generalized varicelliform rash in an otherwise well newborn. No signs of organ involvement were found on repeated skeletal radiography, abdominal ultrasonography and laboratory studies. A diagnosis of "pure cutaneous" LCH was established. Skin manifestation was unusually severe and recurred during the first 20 months of life, but responded well to combination chemotherapy (methylprednisone, vinblastine) while the child continued to thrive. At the age of 2 years the patient presented with acute onset diabetes insipidus due to infiltration of the hypothalomo-pituitary stalk region. He died for reasons unknown at the age of 28 months.
Conclusion: "Pure cutaneous" LCH, frequently also referred to as congenital self-healing LCH, is a variable disorder which may be complicated by late organ involvement. Close follow up and thorough diagnostic evaluation is therefore mandatory.