The syndrome is characterized by premature ageing with sclerodermatous skin changes, subcutaneous calcification, short stature, prematurely aged facies, premature arteriosclerosis, diabetes mellitus and a predisposition to malignancy.
Clinical features: The cutaneous features of Werner's syndrome include sclerodermatous skin changes, particularly on the face and extremities, and subcutaneous calcification.
Objective: We aimed to evaluate the clinical features of sclerodermatous chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (AHSCT).
We assessed age, sex, pretransplant diagnosis, conditioning regimen, GVHD prophylaxis, and occurrence of acute GVHD (aGVHD), chronic lichenoid and chronic systemic GVHD, and clinical properties of sclerodermatous GVHD.
4%) of the 19 patients with generalized sclerodermatous changes.
Conclusion: Sclerodermatous GVHD has a late onset and may be quite disabling.
Key words: Allogeneic hematopoietic stem cell transplantation, sclerodermatous graft-versus-host disease