Adult-Type Dermatomyositis with Secondary Lymphoid Follicles Harbouring Reactive B-cells Component

Dermatomyositis (DM) is an immune-mediated inflammatory disease characterized by lesions in skin and muscle that are populated by lymphocytic infiltrates. It rarely presents with ectopic lymphoid structures (ELS), as other autoimmune and chronic inflammatory diseases. We describe a case of a 47-year-old male, who presented clinically with proximal muscle weakness, skin rash and elevated creatin kinase (CK) level. The muscle biopsy revealed inflammatory necrotizing myopathy, perifascicular pathology, and scattered ectopic lymphoid follicles-like structures (ELS) that was harboring reactive B-cells component. Clonality analysis of B-cell populations using polymerase chain reaction (PCR) has ruled out malignant lymphoma. The patient responded favorably to the steroid therapy, and his muscle weakness improved. Conclusively, the clinical and histopathologic features of DM can be atypical, and the presence of ELS is not inevitably linked to an unfavorable prognosis.

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