History
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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