Eosinophilic perimyositis as the presenting feature of a monoclonal T-cell expansion.
A 51-year-old physically active man was investigated for exertional myalgias and muscle stiffness. On examination he had mild proximal muscle weakness of the upper extremities and retraction of the digit flexors. Blood eosinophilia was present, but serum creatine kinase (CK) levels and an electromyographic study were normal. A skin-fascia-muscle biopsy of the calf revealed a macrophagic and CD4+ T-cell infiltration of the perimysium, and a T-cell expansion was observed in blood, bone marrow, and muscle. A diagnosis of eosinophilic perimyositis was made, and prednisone and azathioprine were administrated with a good clinical response. This case highlights the differential diagnosis of blood eosinophilia with muscle disorders, and underscores that eosinophilic perimyositis may be the expression of a T-cell monoclonal expansion. Although the pathogenesis behind the T-cell expansion is unclear but probably inflammatory, we suggest regular follow-up to allow early treatment of any T-cell lymphoproliferative malignancy that may develop.
Dunand
Murielle
M
Neurology Service, CHU Vaudois, Room BH07/306, 1011 Lausanne, Switzerland.
Lobrinus
Johannes A
JA
Spertini
Olivier
O
Kuntzer
Thierry
T
D001132:Arm / Q000503:physiopathology; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology; D002999:Clone Cells / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D016603:Eosinophilia-Myalgia Syndrome / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D004804:Eosinophils / Q000473:pathology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008264:Macrophages / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology*; D009220:Myositis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*