title: [Localized scleroderma (morphea) in childhood].
abstract: Localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extracutaneous symptoms and antinuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or UVB-narrow band) is suitable for adolescents with superficial circumscribed subtypes.
authors.affiliation: Abteilung für Pädiatrische Dermatologie, Kinderspital Zürich, Steinwiesstr. 75, 8032 Zürich, Schweiz. lisa.weibel@kispi.uzh.ch
authors.forename: L
authors.initials: L
authors.lastname: Weibel
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D003879:Dermatologic Agents / Q000627:therapeutic use; D006801:Humans; D008727:Methotrexate / Q000627:therapeutic use*; D010789:Phototherapy / Q000379:methods*; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D003879:Dermatologic Agents; D008727:Methotrexate