title: Nephrotic syndrome revealing a disseminated Langerhans' cell histiocytosis.
abstract: 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.
authors.affiliation: Department of Nephrology, Ibn Rochd Hospital, Casablanca - Marocco.
authors.forename: Abdelkabir
authors.initials: A
authors.lastname: Anabi
authors.forename: Faissal
authors.initials: F
authors.lastname: Tarrass
authors.forename: Khadija
authors.initials: K
authors.lastname: Hachim
authors.forename: Rachid
authors.initials: R
authors.lastname: Aghai
authors.forename: Mohamed G
authors.initials: MG
authors.lastname: Benghanem
authors.forename: Benyounes
authors.initials: B
authors.lastname: Ramdani
authors.forename: Driss
authors.initials: D
authors.lastname: Zaid
authors.forename: Hicham
authors.initials: H
authors.lastname: El Attar
authors.forename: Leila
authors.initials: L
authors.lastname: Badr
authors.forename: Saida
authors.initials: S
authors.lastname: Sqalli
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006801:Humans; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000473:pathology; D012074:Remission Induction; D013154:Spleen / Q000473:pathology; D013156:Splenectomy
chemical_list: D005938:Glucocorticoids