Long-Term Follow-Up of A Patient with Juvenile Dermatomyositis and Degos-Like Disease

Abstract

Background: Degos-like disease has been associated with several rheumatologic conditions such as systemic lupus erythematosus, antiphospholipid syndrome, systemic sclerosis and rheumatoid arthritis. Case presentation: Seven-year-old girl with low weight, height and body mass index who presented with swollen eyelids with ptosis, suggestive cutaneous features of juvenile dermatomyositis along with the presence of scarce papules with hematic crusts and white atrophic scars with an erythematous rim (Degos-like), dysphagia, flaccid paralysis and proximal and distal weakness. With the use of several immunosuppressants the disease was controlled; and after 7 years of follow-up the patient’s condition remains stable.Conclusion: Herein we describe a unique case of juvenile dermatomyositis associated with Degos-like disease responsive to treatment and a long follow-up.