Clinically amyopathic dermatomyositis (CADM) is characterized by skin manifestations with minimal to no muscle involvement. It is a unique subset of dermatomyositis, which may create a diagnostic challenge due to its vague presentation. Establishing the diagnosis is crucial as CADM is highly associated with rapidly progressive interstitial lung disease (RP-ILD), and patients who suffer from thereof have an abysmal prognosis. Herein, we described a case of a 46-year-old male who presented with a history of skin rash and then started to experience shortness of breath. His respiratory symptoms were progressing swiftly and affected his daily life activities. The initial blood tests were normal, but his chest imaging revealed fibrotic nonspecific interstitial pneumonia. The patient required intubation due to a critical respiratory condition, and later, he needed extracorporeal membrane oxygenation (ECMO). While the patient was connected to an ECMO machine, a bedside open lung biopsy (BOLB) was performed, and the results were in keeping with RP-ILD and CADM. The patient was started on cyclophosphamide without a response, and his chest computed tomography showed acute respiratory distress syndrome. His hospital course was complicated with pneumonia, severe kidney dysfunction requiring dialysis, and candidemia, which resulted in the patient's death.
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http://dx.doi.org/10.7759/cureus.27839 | DOI Listing |
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de São José, Lisbon, PRT.
Anti-melanoma differentiation-associated protein 5 (anti-MDA5) clinically linked amyopathic dermatomyositis (CADM) is a rare autoimmune condition strongly linked to rapidly progressive interstitial lung disease (RP-ILD), a life-threatening complication. We present a 63-year-old female patient with anti-MDA5-positive CADM, who developed RP-ILD with an imaging pattern consistent with organizing pneumonia. She presented with Gottron's papules, periungual erythema, progressive dyspnea, and anorexia.
View Article and Find Full Text PDFEur J Case Rep Intern Med
November 2024
Department of Lung Diseases and Thoracic Surgery, Pauls Stradins Clinical University Hospital, Riga, Latvia.
Background: Clinically amyopathic dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis often linked with the presence of autoantibodies targeting melanoma differentiation-associated protein 5 (MDA5). Patients with CADM are at increased risk of developing rapidly progressing interstitial lung disease, which significantly increases both morbidity and mortality compared to other forms of inflammatory myopathies. While there is no standardized treatment regimen, current therapeutic strategies are generally focused on combination immunosuppressive therapies.
View Article and Find Full Text PDFSAGE Open Med Case Rep
December 2024
Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, AB, Canada.
Dermatomyositis (DM) is an autoimmune idiopathic inflammatory myopathy with characteristic dermatologic manifestations. Myositis-specific autoantibodies (MSAs) delineate DM subtypes and their prognoses. Uncommonly, patients present with distinct clinical features of DM, including photosensitive dermatitis, heliotrope rash, Gottron's papules, and nailfold changes; however, their autoimmune serology is negative for expected MSAs.
View Article and Find Full Text PDFMod Rheumatol Case Rep
December 2024
Department of Rheumatology, Tohoku University Hospital, Sendai, Japan.
The skin ulcers sometimes appear in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM), and usually associates with disease activity. Here, we report a case of 41-year-old woman with anti-MDA5 antibody-positive DM, who developed refractory skin ulcers during the remission induction therapy, which were proven to be associated with clinically silent Staphylococcus aureus bacteremia with septic thrombi in her lung. The patient was referred to our hospital for the treatment of amyopathic DM with interstitial lung disease.
View Article and Find Full Text PDFAfr J Thorac Crit Care Med
October 2024
Department of Medicine, School of Clinical Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
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