Langerhans cell histiocytosis in monozygotic twins

Langerhans cells histiocytosis, one of a group of histiocytosis syndromes characterized by Langerhans cell infiltration, has many clinical manifestations. In the past 30 years, numerous cases of presumed Letterer-Siwe disease, the acute multiorgan variant, have been reported in twins and siblings. Only recently has the Histiocyte Society established a criterion for a "definitive diagnosis" of Langerhans cell histiocytosis--the presence of Birbeck granules within the cells of the histiocytic infiltrate. We report the fatal outcome of Langerhans cell histiocytosis in monozygotic twin infants. There is no satisfactory explanation why Langerhans cell histiocytosis occurs concurrently in twins. We suggest that cytokines may provide an endogenous signal that triggers the pathologic proliferation of Langerhans cells.