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


Langerhans cell granulomatosis is an uncommon disorder of uncertain etiology. It usually affects skin, bone, and the pituitary gland and may also affect the lungs, intestines, liver, spleen, bone marrow, and brain.[6] The disease can be unifocal or multifocal. Clinically, the most common sites of osseous LCG are the skull and proximal femur.[4,6] The most common extraosseous sites involved at time of diagnosis include the skin, lung, and lymph nodes. Our case is the first demonstration of LCG in the hip synovium. The literature contains one other case involving the synovium of the shoulder, which presented itself as tenosynovitis.[7] Our patient initially had hip pain due to villous synovial proliferation. Histologically, our case demonstrated polypoid villous masses lined by synovial cells and containing nodular collections of histiocytes, with scattered eosinophils and giant cells. The histiocyte-like cells by immunohistochemical study proved to be CD1a and S100 positive dendritic cells of the Langerhans phenotype. Within 21/2 months after diagnosis, our patient presented classical multifocal LCG with skin and abdominal lymph node involvement. Involvement of lymph nodes is a common finding in cases of LCG.[8] The LC can involve lymph nodes as sinusoidal, nodular granulomatous collections, and total effacement patterns.[8]

Dendritic-cell—related pathologic diseases purportedly include interdigitating dendritic cell sarcoma (DCS), follicular DCS, congenital solitary reticulohistiocytosis, MRH, and Langerhans cell granulomatosis.[9,10,11,12,13,14,15,16,17,18] Dendritic cell sarcoma and follicular DCS can easily be distinguished from LCG by phenotype. Follicular DCS is CD25 and CD35 positive and CD1a and S100 negative. Dendritic cell sarcoma is CD25, CD1a, and CD35 negative and S100 positive. These two diseases usually produce histiocytic-like/fibroblastic tumors within organs that they normally infiltrate, such as spleen, liver, and more commonly lymph nodes. Congenital self-healing or solitary reticulohistiocytosis is a congenital benign disease restricted to the skin.[11,12] Histiologically, these lesions are composed of histiocyte-like cells and giant cells. The solitary reticulohistiocytosis cells are phenotypically identical to LCG. Solitary reticulohistiocytosis usually spontaneously resolves without further progression. Multicentric reticulohistiocytosis is a rare systemic cutaneous-articular disease that has the closest relationship to our case.[14,15,16,17,18,19] Multicentric reticulohistiocytosis commonly affects the synovial lining of long bones producing destructive nonpannus-like erosive lesions. Unlike LCG, in most cases of MRH, articular symptoms precede skin lesions. Articular manifestations of MRH are similar to those of rheumatoid arthritis: bilateral symmetric involvement, with joint erosions. Histologically, the skin and synovial lesions are characterized by a proliferation of histiocyte-like and multinucleated giant cells. Not all reports of MRH concur regarding the cell of origin. Some reports show the histiocyte-like cells in these lesions to be similar to dermal dendritic cells (that is, positive for CD1c, S100, and factor XIIIa and negative for CD1a,[14] whereas others identify monocyte/phagocyte markers HAM56 and CD68 in these lesions.[15,16,18] Most reports to date, however, agree that the MRH cell is not of true Langerhans phenotype. Unlike LCG, Birbeck granules are ultrastructurally either less numerous, absent, or transformed into laminar bodies, suggesting a more intense cytokine secretory activity in this disease.[20]

In summary, we have reported a rare case of LCG manifested as primary polypoid hip synovial proliferation. The aggressive features of this case were further shown by its eventual systemic dissemination after 1 year, with final control of its growth by chemotherapy. Review of the literature indicated this is a rare occurrence. We conclude that with proper clinical, radiologic, histologic, and immunocytochemical data, the separation of LCG from true monocytic/histiocytic and dendritic cell proliferations can be made.


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