Palatal Telangiectasias as an Ominous Sign of Refractory Dermatomyositis!

A 20-year-old male presented with fever, rash, and proximal muscle weakness of 3 weeks duration. On examination, along with classical cutaneous features of dermatomyositis like heliotrope rash, malar rash, and Gottron papules (Figure 1A,B), we also noticed palatal telangiectasia (Figure 1C). His proximal muscles of the upper limbs and lower limbs had a power of 2/5, and neck muscles were weak. There were no symptoms or signs suggestive of overlap with other connective tissue diseases. His investigations revealed elevated acute phase reactants, transaminitis (AST—513 U/L > > ALT 89 U/L), with markedly raised creatinine kinase (57 000 U/L) and LDH (1092 U/L). His PET CT showed features of diffuse myositis (Figure 1D). His antinuclear antibody and extended antinuclear antibody tests were negative. His extended 16 antigen myositis antigen panel, including Mi2, TIF1γ, MDA5, NXP2, SAE1, Ku, PMScl (75/100), Jo-1, SRP, PL7, PL12, EJ, OJ, and RO-52 was negative (it can be negative in 35%–45% of cases [1]). He also underwent a muscle biopsy showing mononuclear cell infiltration, perifascicular atrophy with occasional necrosis. He was diagnosed as a case of dermatomyositis and started on intravenous methylprednisolone 1 g/day for 3 days, followed by 1 mg/kg oral prednisolone. However, his clinical condition rapidly deteriorated with respiratory muscle weakness requiring ventilatory support. He was subsequently started on intravenous immunoglobulin(2 g/Kg) and rituximab infusion with minimal improvement. He was started on plasmapheresis as a rescue therapy. After five cycles of plasmapheresis, he showed marked clinical improvement. Apart from classical cutaneous findings [2], we highlight a novel clinical finding of palatal telangiectasia as a clinical sign of severe disease activity in dermatomyositis. In a similar case, gingival telangiectasias have also been described as a cutaneous manifestation of dermatomyositis [3]. We also raise the possibility of a novel myositis-specific antibody in our case. Another take-home message is that plasmapheresis can be used as a rescue therapy in such refractory cases [4]. N.G., J.S., A.J., I.G. manged the case. J.S., N.G. drafted the manuscript. M.G.V., S.G., A.V.S.A.K. reviewed the manuscript. The authors have nothing to report. Written consent has been obtained from the patient's relative for publication. The authors declare no conflicts of interest. The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.