An 8-month-old child was admitted to a paediatric intensive care unit in Guinea-Bissau with severe blistering dermatosis. He was treated with broad spectrum antibiotics and dressings, without improvement. After 2 weeks, linear IgA bullous dermatosis was suspected. Owing to lack of dapsone, the child was treated with prednisolone and improved. To avoid corticosteroids side effects, 2 months after starting prednisolone we switched to colchicine, but the boy's condition worsened for reasons of poor adherence, requiring intravenous corticosteroids and antibiotics. After complete resolution of the skin lesions, we continued with colchicine monotherapy, then changed to dapsone after 3 months. The child did not show any further signs of dermatosis, but his follow-up ended abruptly, because he did not return to the hospital. IgA bullous dermatosis is a challenging diagnosis in settings where pathological studies cannot be conducted. Multidisciplinary treatment is required and colchicine is a good option if dapsone is not available.
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http://dx.doi.org/10.1093/omcr/omz004 | DOI Listing |
Arch Dermatol Res
January 2025
Institute of Social and Political Sciences, Corvinus University of Budapest, Budapest, Hungary.
This study aims to explore the measurement agreement between direct and indirect health utility measures in four chronic dermatological conditions (atopic dermatitis, hidradenitis suppurativa, pemphigus, psoriasis). Outpatients survey data collected between 2015 and 2021 were analysed. Health-related quality of life (HRQoL) outcome measures included time trade-off (TTO), EQ-5D-5L and Dermatology Life Quality Index (DLQI).
View Article and Find Full Text PDFNat Commun
January 2025
National Institute of Health and Medical Research (INSERM) UMRS-976 HIPI, Paris Cité University, Saint-Louis Hospital, 75010, Paris, France.
Endotypes are characterized by the immunological, inflammatory, metabolic, and remodelling pathways that explain the mechanisms underlying the clinical presentation (phenotype) of a disease. Recessive dystrophic epidermolysis bullosa (RDEB) is a severe blistering disease caused by COL7A1 pathogenic variants. Although underscored by animal studies, the endotypes of human RDEB are poorly understood.
View Article and Find Full Text PDFPLoS One
January 2025
Assistant Professor of Health Information Management, Department of Health Information Technology, Ferdows Faculty of Medical Sciences, Birjand University of Medical Sciences, Birjand, Iran.
Minimum Data Set (MDS) enables integration in data collection, uniform data reporting, and data exchange across clinical and research information systems. The current study was conducted to determine a comprehensive national MDS for the Epidermolysis Bullosa (EB) information management system in Iran. This cross-sectional descriptive study consists of three steps: systematic review, focus group discussion, and the Delphi technique.
View Article and Find Full Text PDFZhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2024
Department of Dermatology,State Key Laboratory of Complex Severe and Rare Diseases,PUMC Hospital,CAMS and PUMC, National Clinical Research Center for Dermatologic and Immunologic Diseases,Beijing 100730,China.
Objective To explore the clinical features and treatments of Chinese patients with bullous pemphigoid (BP) induced by immune checkpoint inhibitors (ICI). Methods A retrospective analysis was conducted on 18 Chinese patients with ICI-induced BP treated in the Peking Union Medical College Hospital and 14 Chinese patients with this disease reported in the literature.Furthermore,the research data of non-Chinese patients were used for comparison to outline the clinical features and treatment responses of the Chinese patients.
View Article and Find Full Text PDFViruses
December 2024
Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Via Pansini 5, 80131 Napoli, Italy.
The COVID-19 pandemic has encouraged the rapid development and licensing of vaccines against SARS-CoV-2. Currently, numerous vaccines are available on a global scale and are based on different mechanisms of action, including mRNA technology, viral vectors, inactive viruses, and subunit particles. Mass vaccination conducted worldwide has highlighted the potential development of side effects, including ones with skin involvement.
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