Mysterious Association of Systemic Lupus Erythematosis with Perry Romberg Syndrome

Abstract

Parry Romberg syndrome (PRS), also referred to as progressive hemifacial atrophy, progressive facial hemiatrophy, or idiopathic hemifacial atrophy, was first described by C Parry and M Romberg.¹

          It manifests in the first two decades in morphologically normal-born individuals. It commonly affects one or more dermatomes in the trigeminal nerve territory. It has an early onset of ophthalmic and neurological involvement and a variable maxillo-facial or cardiac involvement. It is an idiopathic, gradually progressive craniofacial asymmetry, following the atrophy of subcutaneous tissue, muscles, osseous, and cartilaginous structures. Ophthalmic involvement occurs in up to 35% of cases². Neurological symptoms manifest in 15 to 20% of cases³.