UK survey of management of paediatric localized scleroderma

Abstract

8 BSPAR107 SCLERODERMATOUS GRAFT VS HOST DISEASE C. McVitty, J. Stevenson, N. Martin, B. Gibson, A. M. Ewins, L. Jones, D. Shanks, C. Grant, M. Berry, J. Duncan and J. E. Davidson Royal Hospital for Sick Children, Glasgow, Royal Hospital for Sick Children, Edinburgh and Raigmore Hospital, Inverness, UK. Correspondence to: C. McVitty. E-mail: catrionamcvitty@googlemail.com Introduction: Cutaneous graft-vs-host-disease (GVHD) is common post-haematopoietic stem cell transplantation (HSCT). Sclerodermatous GVHD is rare, with few paediatric reports. Aims: To describe three paediatric cases of scleradermatous GVHD. Method: Retrospective case note review. Results: A 7-year-old girl was referred 2 years after matched HSCT for ALL. She subsequently presented with severe scleroderma on her legs with reduced range of movement (ROM) of her ankles. The disease progressed, involving her thighs and left arm, with significant joint contractures. She was successfully treated with methylprednisolone, MTX and physiotherapy (PT). A 4-year-old girl was referred 3 years after having two matched HSCTs for MHC class 2 deficiency. She presented with worsening contractures affecting her hands and legs with swelling, erythema and skin tethering. She was successfully treated with PT, occupational therapy (OT) splinting, prednisolone and MTX. A 6-year-old girl with beta thalassaemia major was referred 2 years post-HSCT. Buccal GVHD was noted 4 months post-transplant, treated with steroids and ciclosporin before extra-corporeal photophoresis. Despite initial improvement, hand function deteriorated with reduced ROM and grip strength. She was treated with prednisolone, MTX, PT and OT. Conclusion: Sclerodermatous GVHD is rare in children but results in significant morbidity. In these cases severe joint contractures were seen. Monitoring for early contracture post-HSCT and raising awareness of this potential complication should facilitate earlier diagnosis and better outcomes. Disclosure statement: The authors have declared no conflicts of interest.