Sclerodermatous chronic graft-versus-host disease after allogenic peripheral blood stem-cell transplantation

History and clinical findings: A 46-year-old man presented with suberythrodermia and an acral-accentuated sclerosis, which had been progressing over the past 6 months, with extensive, painful ulcers within the sclerotic areas of the calf. Due to acute myelotic leukemia (AML), an allogenic peripheral blood stem-cell transplantation with subsequent immunosupression with mycophenolatmofetil (MMF) and ciclosporin A had been performed 8 years previously. The patient had discontinued treatment on his own after about 2 years, having suffered a cerebroischemic insult in the meantime. Investigations: Histological examinations revealed sclerodermatous changes. Titres of antinuclear antibodies were unremarkable. Laser-Doppler-flowmetry also indicated an active inflammatory and sclerosing process. FACS analysis of the peripheral blood did not reveal signs of AML recurrence. Diagnosis, therapy and course: The histological pattern in conjunction with the anamnesis indicated a cutaneous chronic graft-versus-host disease (GvHD). No further organ involvement was observed. The MMF therapy which the patient had discontinued was restarted. In addition, PUVA therapy was initiated. These measures and intensive physiotherapeutic exercises in parallel prevented further progression of the sclerosis and secondary mobility limitations. The ulcers healed completely with pentoxifylline and anti-infective treatment. Conclusion: After stem-cell transplantation, early diagnosis of GvHD is especially important due to possible irreversible sclerodermatous changes and other organ manifestations. Also for this reason, strict clinical follow-up is especially important with respect to compliance and efficacy of the immunosuppression.