Bullous periorbital oedema in anti-p155/140-positive dermatomyositis: a case series.

Abstract

Dear Editor, Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by immune-mediated skin and muscle injury. There is a diverse spectrum of clinical phenotypes within DM, and this clinical heterogeneity can challenge timely recognition and diagnosis [1]. Myositis-specific antibodies are increasingly utilized to facilitate recognition of DM subgroups, and provide important insights into clinical phenotypes, prognosis and treatment response [1–3]. Anti-p155/140 (TIF-1γ) antibody is associated with increased malignancy risk, refractory disease and distinct cutaneous lesions [e.g. verruca-like palmar papules, hypopigmented and telangiectatic (‘red on white’) patches, and an erythematous ovoid palatal patch] [3, 4]. Herein, we describe five cases of DM with bullous periorbital oedema identified at the Johns Hopkins Myositis Center, all of whom were positive for anti-p155/140, suggesting that this may represent a novel clinical manifestation of this subgroup. A 76-year-old white female presented with a 6-month history of periorbital oedema, proximal muscle weakness and dysphagia (Supplementary Table S1, available at Rheumatology online). Bullous periorbital oedema with heliotrope rash (Fig. 1A), non-scarring alopecia and proximal muscle weakness were noted. Creatinine kinase (CK) was mildly elevated (303 U/l: upper limit of normal 182 U/l). Aldolase was normal (7.9 U/l: upper limit of normal 8.1 U/l). Anti-p155/140 was positive. EMG showed non-irritable myopathy, while MRI noted extensive intramuscular and fascial oedema involving the pelvic and thigh muscles. Bullous oedema and weakness resolved with glucocorticoids, MTX and IVIG (Fig. 1B).