Successful Treatment of Anti-MDA5 Dermatomyositis Associated Cutaneous Digital Pulp Ulcerations With Hyperbaric Oxygen Therapy.

Abstract

DESCRIPTION A 67-year-old male presented with an upper extremity eczematous dermatitis, isolated painful papules on his palmar and dorsal fingers, symmetric inflammatory polyarthritis, Raynaud's disease, and weight loss. He developed painful distal finger ulcerations with surrounding purpura involving the digital pulp (Fig. A and B). Laboratory evaluation revealed normal levels of creatinine kinase and aldolase and a positive melanoma differentiation-associated gene 5 (MDA5) antibody consistent with anti-MDA5 amyopathic dermatomyositis. High-resolution computed tomography of the chest revealed peripheral and peribronchial ground glass opacities and pulmonary function tests were normal. A malignancy workup was unremarkable. Treatment was initiated with prednisone, mycophenolate mofetil, and hydroxychloroquine. Raynaud's was treated with nifedipine initially, followed by sildenafil, bosentan, and topical nitroglycerin. IV Immunoglobulin was attempted but was complicated by a pulmonary embolism after his second treatment requiring discontinuation and anticoagulation with apixaban for 2 months. He continued to develop painful new ulcers and progression of digital ischemia, so hyperbaric oxygen therapy (HOT) was requested. He received 37 hyperbaric oxygen sessions delivered at 2.0 atmospheres absolute or 33 feet of seawater over the course of 54 days. At the end of his treatment course his pain had dramatically improved and the ulcerations were nearly healed (Fig. C and D). His hands remained lesion free at his 9-month follow-up. Anti-MDA5 dermatomyositis is a distinctive subtype associated with unique mucocutaneous features, a higher rate of amyopathic or hypomyopathic presentation, and interstitial lung disease (ILD). Painful cutaneous ulcerations involving the digital pulp or periungual regions develop inmore than 80%of patients. They are thought to be caused by an underlying vasculopathy, severe interface dermatitis, vasculitis, or some combination of these factors.