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

Clinical Images: Degos‐like lesions in association with NXP‐2 dermatomyositis 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png ACR Open Rheumatology Wiley

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

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References (6)

Publisher
Wiley
Copyright
© 2023 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.
eISSN
2578-5745
DOI
10.1002/acr2.11570
Publisher site
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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

Journal

ACR Open RheumatologyWiley

Published: Sep 1, 2023

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