Departments of Rheumatology and 1 Medicine and Dermatology, SUNY at Stony Brook and 2 Radiology, Hospital for Special Surgery, New York, NY, USA
Correspondence to: S. Gupta, T16-040 Health Science Center, SUNY at Stony Brook, Stony Brook, NY 11794, USA. E-mail: sandeepgupta_98{at}yahoo.com
SIR, We submit a case with progressive ankle pain and swelling. A biopsy revealed Rosai-Dorfman disease. The use of various immunosuppressive therapies, including anti-TNF therapy with infliximab resulted in no response. The skeletal manifestations of Rosai-Dorfman disease are reviewed.
A 64-yr-old female was seen with left ankle pain and swelling for 68 months. The pain was exacerbated by weight-bearing. The pain followed a relapsing recurrent course with worsening over this period. There was no history of trauma, fevers or tick bites. No other joint was involved. Examination of the left ankle revealed a mild, boggy swelling between the Achilles tendon and medial malleolus and to a lesser extent over the lateral malleolus. Laboratory tests revealed a normal angiotensin-converting enzyme level and RF and Lyme serological tests were negative. A radiograph of the left ankle was normal and MRI revealed a well-circumscribed lesion with diminished signal intensity in both the dome and anterior process of the talus. The lesion extended beyond the medial cortex of the talus into adjacent soft tissue. The distal tibia and fibula were normal. There was a small effusion in the ankle joint.
At this stage the patient was lost to follow-up. She was evaluated at another facility, where she was treated with antibiotics for a prolonged period for presumed chronic osteomyelitis. The patient was evaluated again approximately 4.5 yr later with continued left ankle pain. She had no constitutional features or involvement of other joints. Examination revealed a warm, moderately swollen ankle with tenderness and palpable soft tissue and bony swelling. A repeat MRI showed multiple lesions replacing the normal marrow signals of the talus, navicular and calcaneous. The lesions extended into adjacent soft tissue and the ankle joint.
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A third MRI of the left ankle showed infiltration and replacement of normal marrow involving most of the calcaneous, the entire talus, the navicular and portions of the cuboid and lateral cuneiform with associated lobulated soft tissue masses. The patient was sent for a positron emission tomographic scan, which showed foci of hypermetabolic uptake in multiple sites, including the hindfoot, right lower back superficially, left paraspinal area, left shoulder and supraclavicular area. She was referred to a radiation oncologist for further treatment.
This is the first described case of Rosai-Dorfman disease presenting as chronic monoarthritis of the ankle. It also illustrates the progressive nature of the disorder, as monitored by detailed MRI. This is also the first published use, to our knowledge, of infliximab in this disorder. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare histiocytic proliferative disorder defined histologically by infiltration of histiocytic macrophage cells with typical immunohistochemical features [1, 3, 7]. The disorder usually presents with fever, lymph node enlargement, neutrophilia, high ESR and polyclonal hypergammaglobulinaemia. Extranodal sites commonly involved include skin, upper respiratory tract and bone. A review of all published cases with this disease documented 33 cases of osseous involvement out of a total of 423 cases [1]. Radiologically, the lesions are typically osteolytic [1, 6]. Histologically, compared with lymph nodes there is less phagocytosis of lymphocytes and more fibrosis [2]. The course of the disease is often variable and prolonged, with remissions and exacerbations [1, 2]. The prognosis is best correlated with the numbers of nodal groups and extranodal sites involved. Involvement of the kidney, liver or lower respiratory tract is associated with a worse prognosis. The outcome is usually good, treatment being needed only for involvement or compression of the vital organs. The treatment options include steroids, radiotherapy, chemotherapy, surgery [3, 4], interferon [5] and thalidomide [8]. In a review, the course of untreated Rosai-Dorfman disease not involving or compressing vital organs (40/80 cases) was found to be favourable, approximately 83% of patients having complete remission spontaneously [3]. Of nine patients requiring aggressive therapy who were given radiation therapy, three had complete remission, three had persistent disease and three died. Of 12 patients given chemotherapy, only two (receiving methotrexate and 6-mercaptopurine) had a complete response. Surgery was successful (for the treated site) in eight of nine cases.
In the related disorder of Langerhans cell histiocytosis, an analysis of the in situ cytokines revealed abundant TNF-, suggesting a possible role in its pathophysiology [9]. Etanercept, a drug that neutralizes TNF-
, has been used successfully in a patient with this disease [10].
Our case presented with progressive osseous involvement masquerading as chronic monoarthritis of the ankle joint, and illustrates the need to obtain tissue for proper diagnosis in the setting of chronic monoarthritis.
The authors have declared no conflicts of interest.
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