title: Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease.
abstract: Juvenile scleroderma is a rare connective tissue disease that involves the skin and subcutaneous tissue. Among all presentations of juvenile scleroderma, localized scleroderma (JLSc) is the most frequent, followed by systemic disease (JSSc) and eosinophilic fasciitis (EF). In posttransplantation chronic graft-versus-host disease (GvHD), scleroderma-like skin involvement can occur. Systemic forms of juvenile scleroderma and GvHD can affect the internal organs, such as the lungs, the gastrointestinal tract, the heart, and kidneys and cause disability and severe, sometimes lethal, complications. Here, the authors give an overview of different presentations of juvenile scleroderma. They report their experience with the different forms and presentations of scleroderma, diagnostic workups, treatment, and outcome of all forms of childhood scleroderma in the context of the existing literature.
authors.affiliation: University Hospital Carl Gustav Carus, Technical University of Dresden, Dresden, Germany. christian.hedrich@uniklinikum-dresden.de
authors.forename: Christian Michael
authors.initials: CM
authors.lastname: Hedrich
authors.forename: Barbara
authors.initials: B
authors.lastname: Fiebig
authors.forename: Gabriele
authors.initials: G
authors.lastname: Hahn
authors.forename: Meinolf
authors.initials: M
authors.lastname: Suttorp
authors.forename: Manfred
authors.initials: M
authors.lastname: Gahr
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D018450:Disease Progression; D004802:Eosinophilia / Q000175:diagnosis* / Q000188:drug therapy; D005208:Fasciitis / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000188:drug therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000188:drug therapy