Parry-Romberg Syndrome with En Coup de Sabre

Abstract

Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author's experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. Fat grafting to her atrophic lip had been conducted, and steroid injection had been performed on the indurated plaque of the forehead. Case 2 was a 29-year-old woman who had suffered from right hemifacial atrophy and bilateral en coup de sabre for 18 years. Surgical corrections such as scapular osteocutaneous flap and mandible/maxilla distraction showed unsatisfying results.

Parry-Romberg syndrome: A case report of non-invasive treatment

“…Mazzeo, et al irst reported this syndrome with root atrophy of maxillary and mandibular teeth and pulp stones in irst permanent molars [16]. This patients had the mentioned manifestations and also other various manifestations reported in previous literatures [3,5,10,11,15,16,25,27]. On the affected side, teeth was hypoplastic and had normal morphology but with smaller size.…”

“…Most of the patients can bene it from conservative treatments. Treatments vary from prescribing local or systemic anti-in lammatory drugs to reconstructive and orthognathic surgeries [10,25]. In this paper, we have reported a moderate case of Parry-Romberg syndrome, with no familial history of any syndromes.…”

Linear Scleroderma ‘en coup de sabre’ and Epilepsy: A Case Report

“…In the differential diagnosis, the firstly considered disease is progressive hemifacial atrophy (Parry-Romberg syndrome) characterized by progressive hemifacial atrophy without cutaneous sclerosis [8]. However, many authors believe that both diseases are different clinical variants of the same disease [9]. Additionally, LSCS and Parry-Romberg syndrome coexist in 20-37% of the patients with LSCS diagnosis, and both conditions have similar age of onset and disease course [4].…”

Parry Romberg syndrome: A case report and discussion

“…They pointed out the lack of sufficient literature regarding efficacy and safety of steroids and other drugs used to treat the syndrome. [1920]…”