Congenital Letterer-Siwe Disease with Intrauterine Fetal Death: a Case Report and Review of the Literature

Letterer-Siwe disease is a rare proliferative disorder of the Langerhans cells, usually encountered inpatients under the age of 3 years. We present an unusual case of intrauterine fetal death at the 28th gestational week associated with generalized papular skin eruptions and systemic histiocytic infiltrates characteristic of Letterer-Siwe disease. Histopathologic features of Langerhans cells were further confirmed by immunocytochemistry and electron microscopy. The events occurring in pregnancy which may be worth mentioning are: (1) routine chest P-A view X-ray exposure of the mother 2 days before LMP, (2) the mother was exposed to certain Chinese herbs, in particular, the 101 hair growth solution (A Chinese herb mixture used for alopecia; contents unknown) from assisting her husband in topical applications, and (3) the mother has worked in front of computer monitors 8 hours a day for 10 years.