Lupus erythematosus (LE) is an autoimmune disease that presents either as a systemic (SLE) or an isolated skin disease (CLE). Currently, there is no FDA-approved medication specifically for CLE, and is treated with the same approach as SLE. We present two refractory cases of SLE with severe cutaneous manifestations unresponsive to the first-line therapy treated with anifrolumab. First, a 39-year-old Caucasian female with a known history of SLE with severe subacute CLE presented to the clinic for her refractory cutaneous symptoms. Her current regimen was hydroxychloroquine (HCQ), mycophenolate mofetil (MMF), and s/c belimumab with no improvement. Belimumab was discontinued, and she was started on anifrolumab with significant improvement. Another, a 28-year-old female with no known medical history was referred to a rheumatology clinic for elevated anti-nuclear antibody (ANA) and ribonucleoprotein (RNP) titers. She was diagnosed with SLE, and was treated with HCQ, belimumab, and MMF but failed to produce a reasonably good outcome. Hence belimumab was discontinued and anifrolumab was added instead with significant cutaneous improvement. The treatment spectrum for SLE is wide, which includes antimalarial (HCQ), oral corticosteroids (OCS), and immunosuppressants (Methotrexate-MTX, MMF, azathioprine-AZT). Anifrolumab, a type 1 IFNα receptor subunit 1 (IFNAR1) inhibitor, has been recently approved by the FDA for moderate to severe SLE while on standard therapy in August 2021. Early use of anifrolumab in moderate to severe cutaneous manifestations of SLE or CLE may result in significant improvement in patients.
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http://dx.doi.org/10.7759/cureus.39553 | DOI Listing |
Clin Exp Dermatol
January 2025
St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Background: In recessive dystrophic epidermolysis bullosa (RDEB), complications like oesophageal strictures, hand contractures, cardiomyopathy and cutaneous squamous cell carcinoma (SCC) may develop, necessitating procedures such as oesophageal dilatation (OD), gastrostomy tube placement and hand surgery.
Objectives: To determine prevalence and age of onset of milestone events by RDEB subtype, specifically dysphagia, first OD, first gastrostomy tube, first hand surgery, cardiomyopathy, first SCC and death.
Methods: The Prospective Epidermolysis Bullosa Longitudinal Evaluation Study (PEBLES) is a register study of individuals with RDEB which records comprehensive EB- and non-EB-related health information.
Clin Cosmet Investig Dermatol
January 2025
Department of Dermatology, Guangzhou Dermatology Hospital, Guangzhou, Guangdong, People's Republic of China.
Scleromyxedema (SM) is a rare primary cutaneous mucinosis characterized by systemic papules and scleroderma-like manifestations, often associated with monoclonal gammopathy. We present the case of a 37-year-old male with SM who developed yellowish plaques on the neck and back over three years. Histopathological examination revealed mucin deposition, fibroblast proliferation, and fibrosis, supporting the diagnosis.
View Article and Find Full Text PDFLupus
January 2025
Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente, Hospital das Clínicas HCFMUSP, Sao Paulo, Brazil.
To identify clusters of autoantibodies in a large cSLE population and to verify possible associations between different autoantibody clusters and the following variables: demographic data, cumulative clinical and laboratory manifestations, disease activity, cumulative damage and mortality. A cross-sectional study was performed in 27 Pediatric Rheumatology University centers, including 912 cSLE patients. The frequencies of seven selected autoantibodies (anti-dsDNA, anti-Ro/SSA, anti-La/SSB, anti-Sm, anti-RNP, aCL IgM and/or IgG and LA) were used for cluster analysis using the K-means method.
View Article and Find Full Text PDFJ Cutan Med Surg
January 2025
Division of Dermatology, McGill University, Montreal, QC, Canada.
Clin Cosmet Investig Dermatol
January 2025
Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Lymphomatoid papulosis (LyP) is currently categorized as a primary lymphoproliferative disorder that follows a chronic, recurrent clinical course. The diagnosis of LyP is mainly based on clinical presentation and histopathological correlation. Six subtypes of LyP have been described and recognized, each with different histological features and sometimes distinct clinical presentations.
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