Gerodermia osteodysplastica and wrinkly skin syndrome are rare autosomal recessive disorders. Due to the many phenotypic similarities in these two conditions, it has been proposed that they represent the same disorder. Both conditions are well delineated in the genetic literature, but despite skin involvement being a striking feature, they are rarely reported in dermatology journals. In this report, we describe three Arab children from two consanguineous families who exhibit overlapping features of gerodermia osteodysplastica and wrinkly skin syndrome. All the patients had dysmorphic facial features, wrinkled skin more marked on the hands and feet, hyperextensible joints, intrauterine growth retardation, developmental delay, congenital dislocation of hips, and osteoporosis. Our observations also support the contention that gerodermia osteodysplastica and wrinkly skin syndrome have the same clinical spectrum; however, this needs to be confirmed at the molecular level.
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http://dx.doi.org/10.1111/j.1525-1470.2007.00586.x | DOI Listing |
Sci Rep
January 2025
Department of Dermatology, University of Maryland School of Medicine, 419 West Redwood Street, Suite 235, Baltimore, MD, 21201, USA.
Erythroderma is a severe and heterogeneous inflammatory skin condition with little guidance on the approach to management in cases of unknown etiology. To guide therapeutic selection, we sought to create an immunophenotyping platform able to identify aberrant cell populations and cytokines in subtypes of erythroderma. We performed high-parameter flow cytometry on peripheral blood mononuclear cells (PBMCs) and whole blood of a patient with refractory idiopathic erythroderma, erythrodermic patients with Sézary syndrome and pityriasis rubra pilaris, and healthy controls.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of Dermatology, Venereology and Leprosy, Nital Skin Clinic, Karad, Maharashtra, India.
Cornea
January 2025
Department of Ophthalmology and Visual Sciences, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Purpose: To evaluate the efficacy and safety of intense pulsed light (IPL) combined with meibomian gland expression (MGX) for the treatment of dry eye disease and meibomian gland dysfunction associated with chronic Stevens-Johnson syndrome and toxic epidermal necrolysis.
Methods: This prospective noncomparative interventional study included 29 patients (58 eyes) who underwent 3 sessions of IPL and MGX at 2-week intervals. Subjective symptoms (ocular surface disease index score) and objective dry eye tests: matrix metalloproteinase 9, tear meniscus height, bulbar redness score, tear film lipid layer thickness (LLT), Schirmer I test, conjunctival and corneal staining, meibomian gland loss, MGX score [meibomian gland score (MGS)], and tear break-up time were assessed at the baseline and after 4, 8, and 12 weeks.
BMJ Open
December 2024
INSERM UMR1231 Génétique des Anomalies du Développement (GAD), Université de Bourgogne, Dijon, France.
Introduction: The megalencephaly capillary malformation polymicrogyria (MCAP syndrome) results from mosaic gain-of-function variants. The main clinical features are macrocephaly, somatic overgrowth, neurodevelopmental delay and brain anomalies. Alpelisib (Vijoice) is a recently FDA-approved PI3Kα-specific inhibitor for patients with PIK3CA-related overgrowth spectrum (PROS).
View Article and Find Full Text PDFJ Trop Pediatr
December 2024
Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006, India.
The clinical profile and outcomes of children with chikungunya infection differ from those observed in adults. As there is a paucity of data on chikungunya infection in children, this study aimed to find the clinical course, complications, and mortality rates of chikungunya infection in children. This was a combined retrospective and prospective observational study.
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