Multicentric reticulohistiocytosis: a rare cause of erosive arthropathy of the distal interphalangeal finger joints

Abstract

Background: Multicentric reticulohistiocytosis (MRH) is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy.

Case report: A case of MRH arthropathy, in which the typical nodular skin manifestation of the disease was absent, is described in a patient with a past history of breast cancer and no evidence of recurrent or new malignancy.

Results: Careful clinical and roentgenological evaluation disclosed important clues to differentiate this condition from other more common distal interphalangeal arthritides--namely, osteoarthritis and its "erosive" variant, rheumatoid arthritis, psoriatic arthritis, tophaceous gout, dialysis related hand arthropathy, and from the rarer fibroblastic rheumatism, all of which can be mimicked by MRH. Histopathology showed the characteristic histiocytic and multinucleated giant cell infiltrate with ground glass cytoplasm, and immunohistochemical analysis showed markers evocative of a monocyte/macrophage origin of MRH.

Figure 1

(A) Absence of skin nodules is coupled with deformity and mild swelling resembling Heberden's nodes of the 2nd and 3rd finger DIP joints of the right hand. (B) The corresponding right hand x rays show the presence of large erosions of the 2nd and 3rd DIP joints. Note the markedly enlarged joint space and the absence of heterotrophic new bone formation.

Figure 2

Synovial membrane cellular infiltrate mainly composed of large histiocytes with eosinophilic ground glass cytoplasm, sparse multinucleated giant cells, and fibrosis (haematoxylin and eosin stain).