cqvip:We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma , systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibro sis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the s kin showed a lichen-ruber-like pattern. Despite repeated extensive investigati ons, no autoantibodies were detectable. Some of these findings looked like those described in juvenile dermatomyositis. Finally, it could be demonstrated that the boy showed microchimerism with approximately 1%maternalCD4+lymphocytesinhisperipheralbloodleukocytes. Furthermore maternal cells could be demonstrated in inflamed muscle tissue. So a graft-versus-host -disease-like pathomechanism appears to be likely. Several systemic therapies have been used with limited success to improve the condition including corticost eroids, azathioprine, cyclosporine A and mycophenolate mofetil. A distinct impro vement of erythemas and sclerosis could be achieved by means of low-dose UVA1 p hototherapy which was applied with escalating single doses of 3-12 J/cm2 for 35 consecutive days.
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