[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus].
BACKGROUND
Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance.


METHODS
A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up).


CONCLUSIONS
There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.
Service de Dermatologie, Hôtel-Dieu, Clermont-Ferrand. mdincan@chu-clermontferrand.fr
M
M
D'Incan
F
F
Franck
J
J
Kanold
F
F
Bacin
R
R
Achin
A J
AJ
Beyvin
F
F
Demeocq
P
P
Souteyrand
D003131:Combined Modality Therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008875:Middle Aged; D009230:Myxedema / Q000150:complications* / Q000188:drug therapy*; D017893:Photopheresis; D011239:Prednisolone / Q000627:therapeutic use*; D012074:Remission Induction; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy*
D005938:Glucocorticoids; D011239:Prednisolone