title: Multicentric reticulohistiocytosis with elevated cytokine serum levels.
abstract: Multicentric reticulohistiocytosis (MRH) is an uncommon non-Langerhans cell histiocytosis of unknown etiology. It is a multisystem disorder characterised by a papulonodular skin eruption, mainly in the extensor surfaces, and destructive polyarthritis. Histologically, either cutaneous lesions or the synovium show a dense dermal infiltrate of histiocytes and multinucleated giant cells with an eosinophilic granular material in the cytoplasm. In the immunohistochemical analysis these cells stain positively with monocyte/macrophage markers (CD68 and CD45), as well as with certain cytokines (tumor necrosis factor-α, interleukin 1β and interleukin 6). Moreover, recent reports suggest an osteoclastic nature of the infiltrating cells, as they stain strongly with osteoclast tissue lytic markers including tartrate-resistant acid phosphatase and cathepsin K. We report a case of MRH presenting with clinical features of dermatomyositis. Furthermore, the patient showed elevated cytokine serum levels that lowered after therapy.
authors.affiliation: Department of Dermatology, Hospital Clinic, Barcelona, Spain. tbennassar@hotmail.com
authors.forename: Antoni
authors.initials: A
authors.lastname: Bennàssar
authors.forename: Antoni
authors.initials: A
authors.lastname: Mas
authors.forename: Antonio
authors.initials: A
authors.lastname: Guilabert
authors.forename: Marc
authors.initials: M
authors.lastname: Julià
authors.forename: José Manuel
authors.initials: JM
authors.lastname: Mascaró-Galy
authors.forename: Carmen
authors.initials: C
authors.lastname: Herrero
mesh_terms: D000368:Aged; D016207:Cytokines / Q000097:blood*; D003882:Dermatomyositis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D012871:Skin Diseases / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*
chemical_list: D016207:Cytokines; D011241:Prednisone; D008727:Methotrexate