title: Sclerodermatous chronic graft-versus-host disease--a report of four pediatric cases.
abstract: The sclerodermatous variant of chronic graft-versus-host disease postallogeneic bone marrow transplantation is rare. We present four pediatric cases of sclerodermatous variant of chronic graft-versus-host disease describing their clinical appearance, management, and outcomes. We compare the pharmacologic and supportive therapies administered to these patients with the management suggested in the current literature. Several key findings were noted. There was a significantly higher mortality rate observed in this series compared with previous reports, with three of the four patients dying ultimately as a result of sclerodermatous variant of chronic graft-versus-host disease. The development of widespread ulceration, in two of the four patients, appeared to be associated with an overall deterioration in the clinical condition. In two patients high-dose thalidomide at 12 mg/kg/day seemed to halt the progression of cutaneous disease. Optimal care of sclerodermatous variant of chronic graft-versus-host disease patients required a multidisciplinary team. A lack of community services observed in this case series led to the need for unnecessarily prolonged inpatient admissions.
authors.affiliation: Department of Dermatology, Royal Hospitals Trust, Belfast, United Kingdom. julia.tolland@gmail.com
authors.forename: Julia P
authors.initials: JP
authors.lastname: Tolland
authors.forename: Clare
authors.initials: C
authors.lastname: Devereux
authors.forename: Frank C G
authors.initials: FC
authors.lastname: Jones
authors.forename: E Ann
authors.initials: EA
authors.lastname: Bingham
mesh_terms: D000293:Adolescent; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002648:Child; D002908:Chronic Disease; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D013792:Thalidomide / Q000627:therapeutic use
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide