Case Report

Beneficial Effect of Immunosuppressive Drugs on Parry-Romberg Syndrome: A Case Report and Review of the Literature

Authors: Cengiz Korkmaz, MD, Baki Adapinar, MD, Seval Uysal, MD

Abstract

Progressive facial hemiatrophy, also known as Parry-Romberg syndrome (PRS), is characterized by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissues and fat. Involvement of the central nervous system with impairment of neurologic function occurs infrequently. At present, there is no agreement as to whether PRS is a distinct entity or a clinical variant of linear scleroderma en coup de sabre. The exact reason for PRS has not yet been determined; therefore, no suitable treatment exists. We observed beneficial effects of immunosuppressive agents on neurologic lesions in particular in a patient with PRS who presented with immunoinflammatory findings and neurologic involvement, apart from cutaneous manifestations.


Key Points


* Progressive facial hemiatrophy may have immunoinflammatory features similar to those of linear scleroderma en coup de sabre.


* Immunosuppressive drugs might well be considered in the treatment of at least some eligible patients with Parry-Romberg syndrome, especially in the case of cerebral involvement.

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