Parry-Romberg syndrome in a patient with scleroderma

Abstract

Parry-Romberg syndrome (PRS) is characterised by progressive but self-limiting facial hemiatrophy. We describe a 48-year-old woman with a 3-year history of gradually worsening right facial hemiatrophy on a background of scleroderma. Her initial primary concern was alopecia. Within the last year, there was greater prominence of her right zygoma and hyperpigmentation on her forearms and left neck. She also had worsening headaches and neck stiffness in the mornings. A clinical diagnosis of PRS was made and she was subsequently treated with a course of methotrexate. She is due to be followed up by dermatology, rheumatology and maxillofacial surgery with the aim of reconstructive surgery once her symptoms stabilise.

A case of overlapping adult‐onset linear scleroderma and Parry‐Romberg syndrome presenting with widespread ipsilateral neurogenic involvement

“…However, scleroderma en coup de sabre causes hemifacial atrophy predominantly involving the scalp and forehead, whereas our patient had involvement of the entire left half of her face without facial skin lesions, findings suggesting concurrent PRS. Indeed, such coexistence of linear scleroderma and PRS has repeatedly been reported in the literature …”

“…Because linear scleroderma and PRS share many clinical features, including young age at onset, female predilection, worsening during pregnancy, elevated autoantibody titers, and the presence of skin lesions, all of which were observed in the present patient, many authors have speculated that the two syndromes lie in the same spectrum . Various causes for these syndromes have been proposed, such as infection, radiation exposure, trauma, genetic predisposition, sympathetic nervous system dysfunction, vascular abnormalities, inflammatory conditions, and autoimmune disorders .…”

“…Parry–Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a rare disorder that causes unilateral facial atrophy. Because these conditions present with overlapping manifestations and preferentially affect females in the first 20 years of life, similar pathological mechanisms are assumed . Linear scleroderma occasionally accompanies myositis or myopathy, whereas about one‐fifth of PRS patients have unilateral limb atrophy, mostly ipsilateral to their facial atrophy .…”

Progressive facial hemiatrophy (Parry-Romberg syndrome): short case report

“…It often affects the maxillary and orbital region [4,8]. 20% of patients have neurological symptoms, such as headaches and cognitive impairment, brain atrophy, epilepsy, or hemiplegia [4,9]. In this case report, the patient presented with headaches and epilepsy which severity increased with the patient's growth.…”

“…Alopecia is sometimes described [9]. Some authors have discussed the premium effect of soft tissues retraction on bone growth and asymmetry, but it seems that bone atrophy is also a primary phenomenon rather than a process secondary to soft tissue retraction [13].…”