title: [Disseminated form of Rosai-Dorfman disease. A case report].
abstract: BACKGROUND
  Extranodal involvement in sinus histiocytosis with massive lymphadenpathy (SHML) or Rosai-Dorfman disease is common, seen in 43% of cases. We present a case of disseminated form of SHML with thyroid, renal, bone and lung involvement.
  
  
  METHODS
  A 53-year-old woman presented with cervical lymphadenopathy and a large palpable mass in the submandibular area. A thyroid nodule was palpable. Laboratory data showed an increase of the erythrosedimentation rate and a polyclonal hypergammaglobulinemia. Histopathological examination of a cervical lymph node biopsy showed typical features of SHML. CT scan showed enlargement mediastinal lymph node, mass of the right lung and infiltrative mass in the right renal hilium. Radiographs revealed lytic lesions in the right proximal tibia and left patella. Histopathology of a biopsy from the kidney mass and thyroidectomy displayed typical characteristics of SMHL. The patient was treated by steroids with a markedly regression of the kidney and renal masses.
  
  
  CONCLUSIONS
  Multiple extranodal involvement in SMHL is rare, the prognosis may be poor when lesions are massive and involve vital organs.
authors.affiliation: Service de médecine interne, hôpital La Rabta, 1007, Tunis, Tunisie. benghorbelimed@yahoo.com
authors.forename: I
authors.initials: I
authors.lastname: Ben Ghorbel
authors.forename: H
authors.initials: H
authors.lastname: Naffati
authors.forename: M
authors.initials: M
authors.lastname: Khanfir
authors.forename: M N
authors.initials: MN
authors.lastname: Kchir
authors.forename: K
authors.initials: K
authors.lastname: Mrad
authors.forename: K
authors.initials: K
authors.lastname: Ben Romdhane
authors.forename: M H
authors.initials: MH
authors.lastname: Houman
mesh_terms: D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis*; D006801:Humans; D008875:Middle Aged