Lower limb onset Parry-Romberg syndrome: An unusual presentation of a rare disease

Abstract

Parry-Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and musclewasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophyof right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. Hewas managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up.