Parry-Romberg Syndrome: A Case Study of Two Patients

Abstract

ABSTRACT Introduction Parry-Romberg syndrome is a rare neurocutaneous disorder, i.e., characterized by a slow and progressive atrophy of facial tissues, including muscles, bones, and skin of one or occasionally both sides of the face. Atrophy may be preceded by cutaneous discoloration of the affected skin, such as hyperpigmentation or depigmentation. Observation A 30-year-old female patient came with a chief complaint of progressive deformity of left-side face since 7 to 8 years. Progressive atrophy on left half face was noticed first at the age of 23 years as an area of hyperpigmentation on left malar region, which progressed with atrophy of the underlying fat and subcutaneous tissue resulting in disfigurement. Head and neck examination confirmed the asymmetry with flattening and hyperpigmentation on left side of face. There was an evident loss of underlying fat, muscle, and subcutaneous tissue giving a sunken appearance. Computed tomography scan revealed generalized atrophy of the soft tissue of the left hemi face resulting in asymmetry on the left side with deviated nasal septum. Another female patient of 35 years old came to the Department of Otorhinolaryngology with marked facial asymmetry on right side. Conclusion Two cases of PRS from different families are discussed. A 30-year-old female who presented with chief complaints of left side hemifacial atrophy and blackish pigmentation of overlying skin whose clinical features were consistent with PRS and a 35-year-old female with right-sided facial hemifacial atrophy. How to cite this article Chandra M, Saxena S. Parry-Romberg Syndrome: A Case Study of Two Patients. Clin Rhinol An Int J 2017;10(1):14-16.