Nephrotic syndrome revealing a disseminated Langerhans' cell histiocytosis

Langerhans' cell histiocytosis (LCH) is a clonal disorder of proliferating histiocytes, which can affect various organs. The clinical picture ranges from localized to disseminated disease with multiple organ involvement and severe organ dysfunction. Renal involvement is uncommon and the association with glomerulonephritis is unusual. We report here a case of nephrotic syndrome caused by membranoproliferative glomerulonephritis (MPGN) in a 20-year-old woman, which revealed a disseminated form of LCH. Surgical removal of the LCH process and high doses of corticosteroids led to the remission of the basic disease and the renal involvement. The occurence of membranoproliferative glomerulonephritis in the course of LCH might be mediated by circulating immune complexes, composed at least in part of antigens or neoantigenes which may cause the abnormal proliferation of the Langerhans' cells.