Clinical Images: Degos-like lesions in association with NXP-2 dermatomyositis.

Abstract

The patient, a 32-year-old woman with a history of NXP-2 dermatomyositis, presented to the combined rheumatology-dermatology clinic for evaluation of a 6-month history of painful, enlarging, and draining ulcers on her left thigh. Prior treatment with antibiotics had not resulted in improvement. She also has myositis (based on elevated creatine kinase and aldolase levels and muscle inflammation on magnetic resonance imaging), joint pain, dysphagia, and gastroesophageal reflux disease. Skin examination revealed a 2 × 2 cm ulcer with an overlying hemorrhagic crust and minimal purulence. In the surrounding skin (porcelain-white) atrophic macules with an erythematous rim on a background of reticulated erythematous patches were noted (A). The patient did not have other classic cutaneous findings of dermatomyositis. A punch biopsy of a macule demonstrated lymphocytic vasculitis (B, part 1) with superficial dermal hyalinization (B, part 2), which is compatible with Degos disease. Two years after her diagnosis, ulcerative lesions have abated, and muscle strength significantly improved. The patient is being treated with intravenous immunoglobulins, azathioprine, steroids, aspirin, and topical tacrolimus. She also continues to work with physical therapy. Degos disease is a rare vascular occlusive disorder of unknown etiology. It can present in a benign or malignant form (1). The benign form is confined to the skin and has been reported to be associated with connective tissue disease, including dermatomyositis, systemic sclerosis, and systemic lupus erythematosus (2, 3). When cutaneous manifestations are accompanied by systemic involvement (most frequently gastrointestinal and central nervous system), this is known as systemic Degos or malignant atrophic papulosis. Systemic Degos is associated with high mortality and morbidity, with bowel perforation and cerebral bleeding being the most common complications. In patients with benign cutaneous Degos, it is important to evaluate for connective tissue diseases and to monitor the patient for systemic Degos, which can present months to years after skin involvement (1). Disclosure Form: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.