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.

Keywords: Encoup de sabre; Hemifacial atrophy; Parry-Romberg syndrome.

Fig. 1

Progessive hemiatrophic change on the right side of face (A), unilateral linear sclerotic brownish patch on the right side of forehead (B), soft non-adhesive brownish patch on the right side of the cheek (C), and sclerotic recession on right nasal alae, right upper lip and gingiva (D).

Fig. 2

Histological findings of forehead showed slightly atrophic epidermis and thickened dermis composed of abundant collagen bundles (A: H&E, ×40; B: H&E, ×400).

Fig. 3

Early clinical presentation of case 2 showed gray atrophic patches on the right side of cheek (A) and chin (B), linear dark brownish atrophic vertical plaque on the left side of forehead (C).

Fig. 4

Recent clinical presentation of case 2 showed progressive hemiatrophic change on the right side of face (A, B) and bilateral linear sclerotic tan colored patches on both sides of forehead (C, D).