A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.
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http://dx.doi.org/10.1111/j.1440-0960.2006.00247.x | DOI Listing |
Introduction: Systemic lupus erythematosus (SLE) causes widespread inflammation and damage in affected organs. Severity is determined by the type of organ systems affected and the extent of involvement. SLE occurs in childhood or adulthood and disease severity varies according to age of onset.
View Article and Find Full Text PDFNeurogenetics
January 2025
Department of Neuroscience and Behavioural Sciences, School of Medicine at Ribeirão Preto, University of São Paulo, Bandeirantes Av. 3900, Ribeirão Preto, São Paulo, 14040-900, Brazil.
Neuronal Ceroid Lipofuscinosis 11 (CLN11) is an ultra-rare subtype of adult-onset Neuronal Ceroid Lipofuscinosis. Its phenotype is variable and not fully known. A 21-year-old man was evaluated in our neurogenetic outpatient clinic for early onset complex phenotype, including learning difficulties, cerebellar ataxia, cone-rod dystrophy, epilepsy, and dystonia.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Basis Dis
January 2025
Alzheimer's Disease Genetics Laboratory, School of Molecular and Biomedical Sciences, Faculty of Sciences, Engineering and Technology, The University of Adelaide, North Terrace Campus, Adelaide, SA 5005, Australia.
Sanfilippo syndrome (mucopolysaccharidosis type III, MPSIII) causes childhood dementia, while Alzheimer's disease is the most common type of adult-onset dementia. There is no cure for either of these diseases, and therapeutic options are extremely limited. Increasing evidence suggests commonalities in the pathogenesis of these diseases.
View Article and Find Full Text PDFBiomedicines
November 2024
Graduate Program in Pharmaceutical Sciences, Federal University of Paraná, Curitiba 80210-170, Brazil.
Glycated albumin (GA) serves as a biomarker for short-term glycemic control (2-3 weeks), playing a role in diabetes management. Our goal was to establish reference intervals (RIs) for serum GA, and the ratios of 1,5-anhydroglucitol to GA (AGI) and GA to HbA1c in a Euro-Brazilian pediatric population (10 y, n = 299), adults (43.5 y; n = 290), and pregnant women (26 y, n = 406; 26.
View Article and Find Full Text PDFDiabetes Care
January 2025
Department of Epidemiology and Biostatistics, School of Public Health, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
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