Congenital Langerhans cell histiocytosis resembling a varicella zoster virus infection

We, report on a preterm infant (33rd gestational week) with a varicella-lik e congenital rash, which initially appeared to respond to therapy with acyc lovir. At the age of 3 weeks, lesions were in different stages of evolution and still resembled a varicella tester virus (VZV) infection. However, sin ce proof of VZV infection was lacking and new lesions erupted at the age of 4 weeks, a skin biopsy was performed which revealed a diagnosis of Langerh ans cell histiocytosis. Therapy with prednisone resulted in prompt healing of the lesions. Discussion: Congenital Langerhans cell histiocytosis is rare and symptoms m ay vary substantially from case to case. Like in our observation it may be confused with congenital varicella. In case of congenital skin lesions of u ncertain etiology a skin biopsy should be performed.