Dermatomyosite œdémateuse sévère

Abstract

Edematous dermatomyositis is a rare entity with localized or generalized subcutaneous edema and only 21 cases have been reported in the literature. It is considered to be a severe form of dermatomyositis which needs quick therapeutic decision. We report 2 cases with difficult therapeutic decisions. Two patients aged 23 and 80 years were admitted in hospital for DM with typical cutaneous and muscular involvement without any sign of gravity and which have been treated by steroids: methylprednisolone bolus and prednisone. They both then developed severe edema of the upper limbs as well as worsening of the cutaneous and muscular symptoms with dysphagia. The addition of mycophenolate mofetil and intravenous immunoglobulin has permitted in the case of the first patient the disappearance of the cutaneous symptoms in particular the edema with restitution of the muscular force and withdrawal of the dysphagia and swallowing symptoms. The therapeutic failure for the second patient was due to a refusal of the treatment and a probable paraneoplastic context. Subcutaneous edema localized or generalized must not be confused with periorbital erythematous edema, classically observed in DM, nor with DM with mucinosis. Potential marker of gravity, it is often associated to important muscular weakness and dysphagia. In this case, an aggressive treatment associating corticosteroids, immunosuppressive therapy and intravenous immunoglobulin is necessary.