[Cutaneous ulceration in dermatomyositis : Case reports with positive anti-NXP-2/anti-TIF1-gamma antibody status].

Abstract

Painful ulcerations developed in a 33-year-old woman with anti-NXP-2-positive dermatomyositis in the facial and trunk areas and a 67-year-old woman with TIF1-gamma-positive dermatomyositis on the hands, while undergoing systemic therapy with azathioprine or low-dose methylprednisolone and cyclic administration of intravenous immunoglobulins (IVIG), respectively. In the laboratory workup, the anti-MDA‑5 antibody status remained negative and the creatine kinase (CK) normal in both patients, while histopathological examinations were nonspecific. Intensive topical class 4 corticosteroid therapy and continuation of the immunosuppressive or immunomodulating therapy led to healing of the ulcerative skin lesions. This report aims to raise awareness of the rare occurrence of cutaneous ulceration in anti-NXP-2- or anti-TIF1-gamma-positive dermatomyositis, as ulcerations may be associated with a severe disease course. In both of our patient cases, it is exclusively a dermal manifestation without other complications.