Focal myositis with fasciitis and vasculitis revealing chronic lymphocytic leukaemia

B. Terrier, F. Lavie, C. Miceli-Richard, A. Azria and X. Mariette

Department of Rheumatology, Hospital Bicêtre, Le Kremlin-Bicêtre, France

Correspondence to: X. Mariette, Rheumatology Unit, Hospital Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, France. E-mail: xavier.mariette{at}bct.ap-hop-paris.fr

SIR, Focal myositis is a rare, localized inflammatory pseudotumour of the skeletal muscle, which is not usually associated with malignant diseases. B-cell chronic lymphocytic leukaemia (B-CLL) can be associated with paraneoplastic features, which are generally autoimmune manifestations such as haemolytic anaemia and thrombocytopenia, and more exceptionally connective tissue disease manifestations such as polymyositis. We report a case of focal myositis with fasciitis-associated with B-CLL. To our knowledge, this is the first report of such an association.

A 74-yr-old woman presented with purpura of the lower limbs and monoarthritis of the left elbow. Laboratory tests disclosed a normal white cell count (leukocytes 7.1 x 109/l, neutrophils 5.2 x 109/l, eosinophils 0.07 x 109/l, lymphocytes 1.6 x 109/l), a normal haemoglobin level and a normal platelet count. Erythrocyte sedimentation rate was 38 mm in the first hour and C-reactive protein was 37 mg/l. Renal function hepatic tests, serum lactate dehydrogenase, serum creatine kinase concentration, chest X-ray and echocardiography were normal. Serum electrophoresis revealed hypogammaglobulinaemia (4.4 g/l). Results were negative for rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibody, cryoglobulinaemia, bacterial cultures and viral serology. Results for anti-Jo-1 (histidyl-transfer RNA synthetase), anti-signal recognition particle (anti-SRP), and anti-Mi-1 and anti-Mi-2 autoantibodies were also negative. Skin biopsy showed mononuclear vasculitis, with a negative direct immunofluorescence study.

The clinical course was marked by fever, localized inflammatory oedema and pain in the right arm.

Magnetic resonance imaging showed T2-weighted sequence hypersignals and enhancement following administration of gadolinium to the deltoid and triceps fascia and deltoid muscle (Fig. 1). Venous Doppler ultrasonography did not show thrombosis of the right arm.



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FIG. 1. (A) T1-weighted sequence enhancement following administration of gadolinium to deltoid fascia and muscle. (B) T2-weighted sequence hypersignals of deltoid and triceps fascia and deltoid muscle.

 
Muscle biopsy of the right deltoid muscle revealed severe myositis with fasciitis and vasculitis. Immunohistochemistry gave positive results for CD4 (80% of lymphocytes), CD8 (20% of lymphocytes) and CD68. Very rare cells were positive for CD20.

Computed axial tomography showed multiple supra- and infraphrenic adenopathic features. Bone marrow biopsy revealed an infiltrate of small, mature lymphocytes (CD20+, CD23+, CD5+, CD10).

Flow cytometry revealed a blood clonal population of CD19+, CD20+, CD23+, CD5+, FMC7, and {lambda} monotypic B lymphocytes, compatible with the diagnosis of B-CLL.

Considering focal myositis as a paraneoplastic syndrome, the patient was treated with 60 mg/day oral fludarabine (without any use of steroids) for 5 days every 28 days.

The course was marked by complete resolution of the clinical manifestations after two courses of chemotherapy and reduction of the clonal population of peripheral leukaemic B cells. After six courses of chemotherapy were performed. Four months after the end of chemotherapy, CLL and focal myositis were still in CR, B-CLL and focal myositis were still in complete remission.

The paraneoplastic nature of this uncommon association of focal myositis with fasciitis and B-CLL was determined on the basis of the simultaneous discovery of the two disorders and their parallel course, and the improvement of muscular symptoms with specific chemotherapy but no steroids. Moreover, immunohistochemistry results of muscle biopsy allowed us to exclude CLL muscle infiltration because of the very rare lymphocytes positive for CD20, most being positive for CD4.

There have been rare case reports of association of myositis with B-CLL, but these have concerned exclusively polymyositis and dermatopolymyositis [1–3].

Focal myositis is a very rare and localized inflammatory pseudotumour of skeletal muscle of unknown origin; histological study of it shows severe myopathy with inflammatory infiltrates and alteration of muscle fibres [4, 5]. Focal myositis is not frequently associated with cancer, since only three cases of localized nodular myositis associated with Hodgkin's lymphoma, non-Hodgkin's lymphoma and phaeochromocytoma have been described [6–8]. Fasciitis, without considering eosinophilic fasciitis, is more frequently associated with neoplasms [9]. Although focal myositis is considered by some authors to be a localized form of polymyositis, there has been very little description of the paraneoplastic feature of this form, and monitoring for neoplasm should be encouraged even in the absence of evolution of the disease towards systemic polymyositis.

Quantitative and qualitative defects of T lymphocytes have been reported in B-CLL and appear to be unrelated to the clinical stage of the disease. Reduced numbers of T-helper cells (CD4+) and increased numbers of CD8 T cells may reflect an appropriate immune response to the malignant cell clone [10]. In our case, the immunophenotype of the peripheral blood lymphocytes showed an inversion of the T-cell CD4:CD8 ratio. Defects of some T-lymphocyte subsets could be the origin of these paraneoplastic manifestations.

Focal myositis with fasciitis may be a paraneoplastic feature of haemopathic conditions, and may respond to specific chemotherapy without any use of steroids.

The authors have declared no conflicts of interest.

References

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Accepted 10 May 2005





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