[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus].
Palabras clave
Abstracto
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.
