Results 51 to 60 of about 1,347 (192)
Treatment of pityriasis rubra pilaris (PRP) may be difficult since no standardized therapeutic approach has been established. Recently, tumor necrosis factor-α (TNF-α) blockers have been demonstrated to be favorable in the management of recalcitrant PRP.
Vito Di Lernia +2 more
doaj +1 more source
Porokeratoses, used here as an example of keratinization disorders, are marked by cornoid lamella and linked to skin cancer risk, making accurate diagnosis essential. This study compared conventional methods, dermoscopy, and histopathology, with advanced imaging using reflectance confocal microscopy (RCM) and multiphoton microscopy (MPM).
Rahime Inci +5 more
wiley +1 more source
Papillon-Lefevre syndrome: A case report with review of literature
Papillon-Lefèvre syndrome is an extremely rare genodermatosis inherited as an autosomal recessive trait, affecting children between the ages of 1-4 years. It has a prevalence of 1-4 cases per million persons.
Rajeev Gadgil +2 more
doaj +1 more source
ABSTRACT Erythrokeratodermia cardiomyopathy (EKC) syndrome is a rare autosomal dominant disorder characterized by generalized erythrokeratoderma and progressive dilated cardiomyopathy, caused by pathogenic variants in the SR6 domain of desmoplakin (DSP).
Sepideh Hamzehlou +7 more
wiley +1 more source
Nagashima-type palmoplantar keratoderma (NPPK) is a common inherited palmoplantar keratoderma predominantly affecting East Asian populations and caused by biallelic loss-of-function variants in the SERPINB7 gene.
Zhenzhen Xiao +3 more
doaj +1 more source
Disseminated punctate keratoderma: a rare case report and review of the literature [PDF]
We report a rare case of a 53-year-old womanpresenting with diffuse, late-onset disseminatedhyperkeratotic papules. Biopsy showed massivehyperkeratosis overlying a crateriform epidermaldepression and hypergranulosis with mild epidermalhyperplasia.
Asemota, Eseosa +3 more
core +1 more source
Mal de Meleda é uma ceratodermia palmoplantar transgressiva rara, com prevalência estimada de 1:100.000 habitantes, descrita em 1826 por Stulli, na Ilha de Meleda.
Fernanda Ayres de Morais e Silva +3 more
doaj +1 more source
Gene for Arrhythmogenic Right Ventricular Cardiomyopathy With Diffuse Nonepidermolytic Palmoplantar Keratoderma and Woolly Hair (Naxos Disease) Maps to 17q21 [PDF]
Background —Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease of unknown etiology that causes arrhythmias, heart failure, and sudden death. Diagnosis can be difficult, and this hampers investigation of its molecular basis.
A S, Coonar +9 more
openaire +2 more sources
Review of Biological Agents in the Therapeutic Management of Monogenic Genodermatoses
Monogenic genodermatoses encompass a diverse group of over 400 distinct disorders, presenting significant therapeutic challenges. Recent advancements in the clinical application of biological agents have heralded a new era in the management of these conditions.
Xueying Wang +4 more
wiley +1 more source
ABSTRACT Mycosis fungoides is a cutaneous T‐cell lymphoma with a classically relatively good prognosis, mostly diagnosed in men over the age of 50. It classically manifests as erythematosquamous plaques, but can take a variety of forms. Here we describe four cases of mycosis fungoides diagnosed in children, with psoriasiform palmoplantar keratoderma as
S. Grandjacquot +7 more
wiley +1 more source

