Clinical features of 24 cases of scleroderma-like cutaneous graft-versus-host disease / 中华皮肤科杂志

ABSTRACT

Objective:To investigate clinical features of and risk factors for scleroderma-like cutaneous graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation.Methods:Clinical data were collected from 24 patients with scleroderma-like cutaneous GVHD in Department of Dermatology, Peking University People′s Hospital from 2014 to 2019. Clinical features, treatment, prognosis, and possible factors influencing the development of scleroderma-like cutaneous GVHD were analyzed retrospectively.Results:Among the 24 patients, 11 were males, and 13 were females, aged 33 ± 12 years; 20 were human leukocyte antigen (HLA) -identical recipients, 4 were HLA-haploidentical recipients; GVHD occurred 18.5 (8.0, 30.9) months after transplantation. Nineteen patients had discontinued anti-rejection therapy or received low-dose anti-rejection drugs before the onset of GVHD. Fifteen patients presented with generalized scleroderma-like lesions, 1 with linear scleroderma-like lesions, 5 with morphea-like lesions, and 3 with fasciitis-like lesions. None of the 15 patients with generalized scleroderma-like GVHD had Raynaud syndrome. Thirteen patients were accompanied by graft rejection in other systems, 8 had joint mobility limitations, and 1 developed cutaneous squamous cell carcinoma secondary to chronic skin ulcers. All patients were treated with systemic glucocorticoids and immunosuppressive agents, and 11 also with topical glucocorticoids. An intensive follow-up was carried out in 11 patients, of whom 3 achieved marked improvement, 4 achieved improvement, 2 experienced exacerbation, and 2 died. A total of 223 patients with non-sclerodermatous cutaneous GVHD admitting during the same period served as controls, and the proportion of HLA-identical patients was significantly higher in the scleroderma-like cutaneous GVHD group (20/24, 83.3%) than in the non-sclerodermatous cutaneous GVHD group (47/223, 21.1%; P < 0.001) . Conclusions:Scleroderma-like cutaneous GVHD commonly occurs late, and can mimic clinical manifestations of all 4 types of spontaneous scleroderma. HLA-identical transplants, premature discontinuation or excessive dose reduction of anti-rejection drugs may be risk factors for scleroderma-like cutaneous GVHD.