Panniculitis as the Transformed Cutaneous Manifestation of Refractory Dermatomyositis with Successful Management with Tofacitinib

Mediterranean Journal of Rheumatology

article

Upendra Rathore¹  Neha Nigam²  Amita Aggarwal¹  Latika Gupta¹ 

[1] Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences;Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences

关键词: panniculitis;    dermatomyositis;    tofacitinib;   

DOI  :  10.31138/mjr.33.3.380

学科分类：社会科学、人文和艺术（综合）

来源: PCO Convin S.A.

【 摘 要 】

A 26-year-old girl presented in 2017 with fever, proximalmuscle weakness, and cutaneous rashes of one-yearduration. A diagnosis of Dermatomyositis (DM) wasmade based on heliotrope and malar rashes, Gottron’ssign, and cutaneous ulcers on the elbows, and significant proximal muscle weakness with a manual muscletesting score of 37/80 with elevated muscle enzymes(AST- 85U/L, ALT-53U/L, LDH-701U/L, CPK-303U/L).Myositis specific antibodies (MSA) were negative. Uponinitiating 1 mg/kg glucocorticoids and methotrexate, arapid improvement in muscle weakness was recordedover 8 weeks though cutaneous disease persisted.Subsequently various drugs (Hydroxychloroquine,Rituximab, Tacrolimus, Mycophenolate mofetil andThalidomide) were unsuccessfully tried for refractorycutaneous disease over the next two years. EventuallyIntravenous Immunoglobulin (IvIg) (2gm/kg 4-weeklydoses) was initiated, following which rashes subsidedand glucocorticoids were tapered and stopped over thenext ten months.

【 授权许可】

CC BY-NC