Sclerodermatous graft‐versus‐host disease: clinical spectrum and therapeutic challenges

• Published in: British journal of dermatology (1951) Vol. 156; no. 5; pp. 1032 - 1038
• Main Authors: White, J.M.L., Creamer, D., Du Vivier, A.W.P., Pagliuca, A., Ho, A.Y., Devereux, S., Salisbury, J.R., Mufti, G.J.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.05.2007; Blackwell
• Subjects: Adult; Aged; Anemia, Refractory - surgery; Biological and medical sciences; Bone Marrow Transplantation - adverse effects; chronic graft‐versus‐host disease; clinical subtypes; Dermatology; Female; graft‐versus‐host disease; Hodgkin Disease - surgery; Humans; Immunosuppressive Agents - therapeutic use; Leukemia, Myeloid - surgery; Male; Medical sciences; Middle Aged; Plasmacytoma - surgery; PUVA Therapy; Recurrence; review; Scleroderma, Localized - classification; Scleroderma, Localized - etiology; Scleroderma, Localized - therapy; sclerodermatous graft‐versus‐host disease; Skin involvement in other diseases. Miscellaneous. General aspects; Stem Cell Transplantation - adverse effects; Thrombocytosis - surgery; treatment; Treatment Failure; Human; Immunopathology; Skin disease; Dermatology; Chronic disease; sclerodermatous graft-versus-host disease; chronic graft-versus-host disease; Graft versus host reaction; Case study; Symptomatology; Treatment; review; clinical subtypes; Sclerodermiform dermatosis; graft-versus-host disease
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1111/j.1365-2133.2007.07827.x

Summary Sclerodermatous graft‐versus‐host disease (GVHD) is a rare complication of bone marrow transplantation. While GVHD is often associated with the beneficial graft vs. tumour effect, it also contributes towards significant morbidity and mortality. No reliably effective treatment has yet been established. We present 10 patients with haematological malignancies who underwent an allogeneic stem cell transplant and developed sclerodermatous GVHD. Donor lymphocyte infusion administered for relapse or reducing donor T‐cell chimerism was a known trigger for sclerodermatous GVHD in four of the patients. Treatment with immunosuppressants, psoralen plus ultraviolet A (PUVA) and extracorporeal photopheresis has been largely unsuccessful in their management. Intensive immunosuppression including the use of anti‐CD20 monoclonal antibody may have contributed to relapse of leukaemia in one patient 10 years after her transplant. Sclerodermatous GVHD may occur without a preceding lichenoid stage. Clinical heterogeneity is common, although sclerodermatous GVHD has a predilection for the limbs. Treatment options are largely unsatisfactory if conventional immunosuppression fails. PUVA may give some symptomatic benefit and extracorporeal photopheresis seems to be less efficacious than previously published work suggests.