Cutaneous Langerhans cell histiocytosis relapsing during pregnancy

Abstract

Langerhans cell histiocytosis is a rare disorder presenting as a single system (monosymptomatic) or multisystem (oligosymptomatic or disseminated) involvement in which various etiopathogenetic theories including inflammatory, reactive or immune dysregulatory ones have been suggested. It is most commonly seen in infancy and childhood. Isolated involvement of the skin was rarely reported in the literature. We describe a case of an otherwise healthy pregnant woman with a disseminated confluent papular eruption on the trunk and extremities with large nodules on the face giving a leonine-face appearance who was diagnosed as cutaneous Langerhans cell histiocytosis without evidence of the systemic involvement.