Clinical Cases

Dramatic Case of Chronic Sclerodermatous Graft-Versus-Host Disease

Dramatic Case of Chronic Sclerodermatous Graft-Versus-Host Disease




A 36-year-old man presented to our clinic with extremely severe sclerodermatous and lichenoid changes of the skin, diffuse hair loss, nail changes and painful oral ulcers. Within the last 2-3 months his skin condition became critical, the chronic pain syndrome turned to be more pronounced and his mobility markedly decreased.

His previous medical history revealed an acute myeloid leukemia that was diagnosed in December 1998. After a high-dose chemotherapy in February 1999, he received allogeneic blood stem-cell transplantation (SCT) from his HLA-identical sister. Since then, he has been in a complete hematological remission. Despite preventive immunosuppressive treatment, three months later a cutaneous graft-versus-host disease (GVHD) developed. Treatment with systemic prednisone, mycophenolate mofetil, tacrolimus, bath-PUVA (70x) and UVA1 (20x) therapy were not effective. Consequently, he developed more severe and extensive chronic sclerodermatous and lichenoid cutaneous changes, followed by impaired joint mobility. He also suffered from alopecia, pronounced sicca syndrome, chronic obstructive lung disease, dysphagia symptoms and chronic pain syndrome. Furthermore, a long-term corticosteroid administration resulted in numerous and serious complications such as osteoporosis, diabetes mellitus, hypertension, behavioral disturbances and caries of his teeth. Additionally, bilateral septic necrosis of heads of humeri resulted in the resection of his left head of humerus in May 2004. The patient also developed bilateral cataracts, and in June 2004 underwent corneal transplantation after a spontaneous rupture of his left cornea. Hypertension and hypercholesterinemia led to atypical angina pectoris and anterior myocardial infarction.

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