%0 Case Reports
%T [Buschke's scleredema successfully treated with extracorporeal photopheresis].
%A Dezoteux F
%A Bourges M
%A Lionet A
%A Carpentier O
%J Rev Med Interne
%V 39
%N 5
%D May 2018
%M 29544653
%F 0.885
%R 10.1016/j.revmed.2018.02.018
%X <B>BACKGROUND: </B>Scleredema adultorum (Buschke's scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients' mobility.<BR><B>METHODS: </B>We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Histologic examination confirmed the diagnosis of scleredema adultorum. The patient was treated with extracorporeal photopheresis (EPP) twice a month for two months. At follow-up, mobility was highly improved after two months. Beneficial effect of EPP was maintained on the long term while sessions were spaced.<BR><B>CONCLUSIONS: </B>EPP is an unconventional treatment of Buschke's scleredema. We described a case of Buschke's scleredema successfully treated with EPP which may represent a therapeutic option for the treatment of scleredema.