CONGENITAL SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS - THE NEED FOR LONG-TERM FOLLOW-UP

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a ra re disorder initially seen at birth or in the newborn period with spon taneously involuting skin lesions. In contrast to other forms of Lange rhans cell histiocytosis in infancy, such as Letterer-Siwe disease, th e prognosis of CSHLCH is generally good. We describe four children wit h widespread CSHLCH at birth. In all patients a congenital infection w as initially suspected. All patients, except one who had transient low er extremity edema and pulmonary symptoms, had an uncomplicated neonat al course, and in all patients skin lesions resolved spontaneously. Tw o patients showed no further evidence of disease, but one had a cutane ous relapse at age 3 months, and in another a bony relapse that requir ed systemic therapy developed at 6 months of age. CSHLCH is usually a benign, self-limited condition, but careful evaluation for systemic di sease must be performed. Long-term follow-up for evidence of relapse o r progression of disease is essential.