45-year-old man presented with a horizontal groove over all his fingernails and toenails. The grooves were most noticeable over his great toenails bilaterally, with a horizontal groove 5 mm from the proximal nailfold (Figure 1). Three and a half months previously, he had been given a diagnosis of coronavirus disease 2019 (COVID-19) after a positive nasopharangeal swab polymerase chain reaction test, owing to symptoms of diarrhea, fever and shortness of breath, which lasted for 10 days and did not require admission to hospital.The cutaneous findings associated with COVID-19 have been classified into 5 distinct patterns: a maculopapular eruption, chilblain lesions, urticarial lesions, a vesicular eruption and livedo reticularis and necrosis. 1 Some of these findings, such as the vesicular eruption, appear early in the disease course; others are seen later, such as the chilblain pattern. 2 Beau lines are transverse grooves in the nail plate that result from transient interruption of the growth of the proximal nail matrix. 3 They are often noticed 2-3 weeks after a systemic insult and their appearance parallels the length of time needed for the nail to grow past the proximal nailfold. As the toenails grow at a rate of about 1.62 mm per month, the distance of the Beau lines from the proximal nailfold in our patient reflects the timing of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. 4 Other causes of Beau lines include localized trauma and systemic triggers such as febrile illness, severe malnutrition, pemphigus, Raynaud disease, Kawasaki disease and chemotherapy. There is no specific therapy for Beau lines, as they selfresolve if the underlying condition has resolved.
Background: Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes. Objective: This purpose of this article is to provide readers with an update on the evaluation, diagnosis, and treatment of juvenile dermatomyositis. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to the English literature. The information retrieved from the above search was used in the compilation of the present article. Methods: A PubMed search was performed in Clinical Queries using the key term “juvenile dermatomyositis” as search engine. Results: Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is high-dose corticosteroid (oral or intravenous) in combination with methotrexate. Conclusion: For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited and their use in the treatment of juvenile dermatomyositis is considered investigational.
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