DOI: 10.36347/sjmcr.2019.v07i07.017

Pages: 455-457

Introduction: Scleromyxedema, or papulosclerotic mucinosis, is a very rare chronic condition, often associated with extracutaneous manifestations. We report a case associated with a unilateral macular edema and treated effectively with melphalan and corticosteroids. Case report: We examined a 57-year-old woman with severe left visual loss and biopsy-proven scleromyxedema disease, by showing mucin deposition in the superficial dermis, diagnosed four months prior to the ocular involvement. The clinical exam showed the presence of skin-colored micropapules, in the face, the ear lobes, the neck and the upper part of the trunk; the face and hands had a sclerodermiform appearance. Meanwhile, the fundus examination found out a unilateral macular edema confirmed with an optical coherence tomography who showed the macular edema with intraretinal cystic spaces and retinal thikening. A monthly course of melphalan (16 mg / day combined with prednisone 2 mg / kg / day for 4 days / month) allowed disappearance of macular edema. And a clear improvement in cutaneous lesions. Discussion: Most information about scleromyxedema has been retrieved from anecdotal or single case reports. scleromyxedema is a chronic disease , with an unpredictable evolution. However, some lucky patients can have spontaneous clinical resolution It can envolve systemic implications, especially neurologic, cardiologic, and hematologic ones, entailing a guarded prognosis. Ocular manifestations are very rare.