[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.
D'Incan
M
M
Service de Dermatologie, Hôtel-Dieu, Clermont-Ferrand. mdincan@chu-clermontferrand.fr
Franck
F
F
Kanold
J
J
Bacin
F
F
Achin
R
R
Beyvin
A J
AJ
Demeocq
F
F
Souteyrand
P
P
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