Results 81 to 90 of about 1,415,478 (254)
A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation. [PDF]
Mice carrying the Tight skin (Tsk) mutation have thickened skin and visceral fibrosis resulting from an accumulation of extracellular matrix molecules.
Buchberg, Arthur M.+7 more
core +3 more sources
Mucosal‐associated invariant T cells in rheumatic diseases.
Mucosal‐associated invariant T (MAIT) cells are innate‐like T cells defined by their semi‐invariant T cell receptor (TCR) and restriction by the MHC class I‐related molecule (MR1). These cells are primarily activated by microbial‐derived metabolites presented by MR1 or by cytokines. Upon activation, MAIT cells rapidly produce pro‐inflammatory cytokines,
Manon Lesturgie‐Talarek+7 more
wiley +1 more source
Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain.
Seval Dogruk Kacar+5 more
doaj +1 more source
Persistent Vascular Collagen Accumulation Alters Hemodynamic Recovery from Chronic Hypoxia [PDF]
Pulmonary arterial hypertension (PAH) is caused by narrowing and stiffening of the pulmonary arteries that increase pulmonary vascular impedance (PVZ). In particular, small arteries narrow and large arteries stiffen. Large pulmonary artery (PA) stiffness
Chesler, Naomi C.+5 more
core +2 more sources
Pansclerotic Morphea with Features of Eosinophilic Fasciitis: Distinct Entities or Part of a Continuum? [PDF]
Scleroderma is a highly complex disorder in its clinical manifestations and pathogenesis. It has a wide range of clinical manifestations due to varying degrees of vasculopathy, autoimmunity, altered endothelium function, and abnormal fibrosis.
Canty, Kristi+2 more
core +1 more source
Ultraviolet A1 (UVA1) phototherapy (spectral range 340‐400 nm) is a well‐established treatment option for various skin diseases such as localized scleroderma. Recent improvements of conventional UVA1 light sources (metal‐halide or fluorescent lamps) have
S. Arndt+5 more
semanticscholar +1 more source
Parry Romberg Syndrome with localized scleroderma: a case report [PDF]
Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome
Gupta, Nikita+3 more
core +1 more source
ABSTRACT Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive heterotopic ossification (HO) and congenital malformation of the great toes. This case describes a 5‐year‐old Caucasian girl who initially presented with painless neck and back swelling as well as severe limitation of movement in the neck and ...
Orhan Yilmaz, Loretta Fiorillo
wiley +1 more source
Clinical Profile of Methotrexate-resistant Juvenile Localised Scleroderma
Methotrexate has demonstrated its efficiency for the treatment of juvenile localized scleroderma but some patients may be resistant. The aim of our study was to define the profile of such patients.
Juliette Hardy+19 more
doaj +1 more source
OBJECTIVE SSc and localized sclerosis (LoS) are considered clinically distinct entities. We describe herein the coexistence of SSc and LoS by both a systematic literature review and an observational cohort study of unselected SSc patients.
A. Vanhaecke+8 more
semanticscholar +1 more source