Langerhans Cell Granulomatosis Manifested as Pigmented Villonodular Synovitis

Cooley G. Pantazis, MD, Kimberly Templeton, MD, Ossama W. Tawfik, MD, PhD, Raul Braylan, MD


J South Orthop Assoc. 2001;10(4) 

In This Article

Abstract and Introduction

We report an unusual case of Langerhans cell granulomatosis (LCG) manifested as a villous synovial proliferation in a 38-year-old female jogger. One year after the onset of joint symptoms, she had a classical LCG presentation with skin and visceral lymph node involvement. Review of the literature revealed only one case of synovial shoulder joint tenosynovitis associated with LCG in a middle-aged woman. Ours is the first reported case presenting clinically in the synovium of the hip joint as pigmented villonodular synovitis. Histiocytic/dendritic proliferations involving the synovial tissues are not uncommon. These lesions as well as the rare multicentric reticulohistiocytosis (MRH), a systemic monocytoid/histiocytic disorder with multinucleated giant cells, polyarthritis, and papulonodular skin lesions, should be considered in the differential diagnosis. Clinical and pathologic features will distinguish LCG from MRH.

Histiocytic infiltration and proliferation within the synovium is a common finding associated with inflammatory processes. The cellular makeup of these monocytoid/histocytic cells is heterogeneous and ranges from cells of lymphoid, histiocytic, and dendritic origin.[1] Antigen-presenting cells or histiocyte-like cells containing Birbeck granules have been identified within inflamed synovial tissues.[2]

Langerhans cell granulomatosis (LCG) is a clonal disorder characterized by lesions containing histiocyte-like cells, giant cells, and eosinophils.[3,4] Langerhans cells are stellate dendritic cells that arise from CD34-positive bone marrow stem cells[5] and are not a member of the mononuclear phagocytic system. Langerhans cell granulomatosis cells are abnormal and coexpress S100 and CD1a, and these markers are used to separate this subpopulation of dendritic cells from other histiocyte-like cells. The pathogenesis of the disease is unclear, but altered expression of cytokines and cellular adhesion molecules, important for chemotaxis of LC, are thought to be involved in its development.[3,6] The biologic behavior of LCG ranges from spontaneous remission to progressive dissemination, and such behavior cannot be predicted on the basis of histologic features.[4]

In the following report, LCG was manifested as a villous synovial proliferation mimicking pigmented villonodular synovitis (PVNS). This is the first reported case with hip synovium involvement. It emphasizes the importance of immunocytochemistry to separate this histiocyte-like cell proliferation from true histiocytic and other dendritic cell types.


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