Eosinophilic fasciitis, myositis and arthritis as early manifestations of peripheral T‐cell lymphoma

Several reports have described an association between eosinophilic fasciitis (EF) and certain infections (1), neoplastic haematologic malignancies, or aplastic anemia (2), but only rarely with T-cell lymphomas (3 – 5). Our case exemplifies the protean rheumatologic manifestations of T-cell lymphoma, and describes unique findings observed by bone scintigraphy. A 56-year-old woman presented with increased fatigue, aches in the shoulders and muscles, and elevated ESR (60 mm/h). Chest X-ray and ultrasound of the abdomen were normal. A diagnosis of polymyalgia rheumatica was made and she was treated with prednisone, 20 mg per day. Her symptoms disappeared, and the prednisone dose was gradually tapered. Six months later she experienced diffuse swelling in her hands and feet, her muscle strength was clearly decreased and a brownish, hard, tender rash appeared on her right calf. Block skin and muscle biopsy showed intense infiltration of inflammatory cells into the muscle, and clearly increased numbers of eosinophils. The most likely diagnostic possibilities considered were eosinophilic fasciitis and eosinophilia – myalgia syndrome. Electromyography revealed myositis limited to proximal paraspinal muscles and mild myopathic changes in the limbs. A magnetic resonance imaging (MRI) study of her lower limbs was nondiagnostic. Serum creatine kinase and aldolase values were normal, as well as the complete blood cell count — including eosinophils. Antinuclear antibodies and antibodies to Borrelia Burgdorferii were negative. The patient was treated with prednisone 40 mg per day with disappearance of arthralgia and skin rash. However, when her prednisone dose was tapered below 10 mg per day the joint pain reappeared and she was referred to the rheumatology unit. At entry she had arthritis in both wrists and left knee, and slight swelling in hands and fingers. Gamma-scan revealed increased uptake in several joints. In proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints the uptake was peculiar: a clear uptake was observed in the growth plates of bones instead of joints. Increased uptake was also observed in the skull, resembling the ‘hot-skull’ phenomenon (Figure 1). Laboratory findings showed that antinuclear and antineutrophil cytoplasmic (ANCA)-antibodies and rheumatoid factor (RF) were negative. Blood eosinophils were slightly increased (0.83|10/L, 12%). Liver enzymes and blood chemistry were normal. C-reactive protein (CRP) was 20 mg/L. Chest X-ray revealed slightly prominent right hilar area and ultrasound of the abdomen revealed a clear lymphadenopathy. Findings were verified by computed tomography (CT)-scan, which also disclosed enlarged lymph nodes in the mediastinum. A biopsy of a cervical lymph node disclosed CD4 nonHodgkin’s peripheral T-cell lymphoma. The diagnosis of peripheral T-cell lymphoma was verifed, and the patient received cytostatic treatment, followed by autologous stem-cell transplantation. Thereafter, she has complained of occasional pain in the muscles, but the skin rash and arthritis have remained absent. The patient presented with typical findings of EF and the relatively good response to corticosteroid treatment also favoured this diagnosis. The first symptoms preceded the appearance of lymphadenopathy by 18 months, and the appearance of EF by approximately 10 months. A diffuse uptake of fascia in bone scintigraphy has been described in EF (6). In our patient a distinct uptake in the growth plates of the small joints of fingers and increased uptake in the skull was observed. This finding could represent a stimulation of the growth plates by cytokines, such as TGF-b (7 – 8), or some other factor secreted by the malignant T-cells. Diffusely increased uptake in the calvarium on bone scintigraphy (a hot skull) has been often described in patients with bone metastases and metabolic diseases (9). This case illustrates the protean manifestations of peripheral T-cell lymphoma. Although in the literature a relative unresponsiveness to glucocorticoids has been described in cases with underlying malignancy in association with EF, it is evident that even a good response to glucocorticoid treatment does not exclude the diagnosis of underlying diseases, such as lymphoma, as in the present case. Close follow-up is therefore warranted after the initial diagnosis.