Congenital “self-healing” Langerhans cell histiocytosis: The need for long-term follow-up

Abstract

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare disorder initially seen at birth or in the newborn period with spontaneously involuting skin lesions. In contrast to other forms of Langerhans cell histiocytosis in infancy, such as Letterer-Siwe disease, the prognosis of CSHLCH is generally good. We describe four children with widespread CSHLCH at birth. In all patients a congenital infection was initially suspected. All patients, except one who had transient lower extremity edema and pulmonary symptoms, had an uncomplicated neonatal course, and in all patients skin lesions resolved spontaneously. Two patients showed no further evidence of disease, but one had a cutaneous relapse at age 3 months, and in another a bony relapse that required systemic therapy developed at 6 months of age. CSHLCH is usually a benign, self-limited condition, but careful evaluation for systemic disease must be performed. Long-term follow-up for evidence of relapse or progression of disease is essential.