China
Africa
Explore chapters and articles related to this topic
Connective Tissue Disorders
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Laura Atzori, Caterina Ferreli, Franco Rongioletti
Management: There is no single evidence-based treatment regimen that is superior for all cases. Topical corticosteroids are the main treatment for plaque-like morphea. Topical tacrolimus 0.1% or calcipotriene are alternative choices. Active extensive morphea or generalized morphea are treated with methotrexate, combination therapy of systemic steroids and methotrexate, or with phototherapy with ultraviolet A1 (UVA1) or narrowband UVB. Linear scleroderma of the face or extremities generally requires the combination of systemic corticosteroids and methotrexate to limit functional disability and/or aesthetic outcomes.
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Children may present with morphoea (round or oval patches of indurated, hyper- or hypopigmented skin), which may become widespread (diffuse form). Linear scleroderma usually affects limbs (upper and/or lower) or face (en coup de sabre) or trunk. The en coup de sabre lesion is associated with uveitis and cerebral involvement (seizures) (Fig. 17.18).
Interstitial Lung disease In Childhood Rheumatic Disorders
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Scleroderma is a rheumatic disease which occurs both in adults and children in systemic and localized forms. Morphea and linear scleroderma are localized forms of disease. It is the generalized disease, progressive systemic sclerosis (PSS), which is associated with severe pulmonary involvement. PSS is characterized by the appearance of Raynaud’s phenomenon, skin thickening and tightness, articular disability and involvement of heart, intestines, kidneys and lungs. Singsen et al.,2 following at least 13 children with PSS, found no significant differences between childhood and adult PSS. The incidence of PSS in the general population is approximately five cases per million people per year.22 In children, onset has been noted as early as 3 years. The female to male ratio is 6:1.4
A case of linear scleroderma “en coup de sabre” with strabismus fixus in a child
Published in Strabismus, 2019
Pradhnya Sen, Easha Ramawat, Amit Mohan, Chintan Shah
Ramboer K et al.5 reported a case of linear scleroderma with MRI findings. They found that the eye was displaced laterally by a band like mass and superior oblique, SR and MR could not be identified, associated features of enophthalmos was also seen. The thickening of orbital muscles seen in their case was hypothesized secondary to orbital fat atrophy and fibrosis.