Linear Scleroderma of the Head - Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties

Abstract

To provide an update on the current management, including evaluation and treatment, and the available diagnostic tools for linear scleroderma of the head, i.e., Parry-Romberg Syndrome and en coup de sabre (PRS/ECDS). A rapid scoping review of the literature was conducted to include manuscripts published in English between 2010 and 2019. Literature searches were performed in PubMed and EMBASE databases. The were analyzed for descriptive statistic reporting. This study reviewed 215 manuscripts reporting these 1430 patients. Surgical reports comprised the majority of the reviewed literature. Most PRS/ECDS did not appear to receive comprehensive multisubspecialty evaluation for extracutaneous manifestations; 21% of cases noted neurological screening, 4% noted dental screening, and 3% noted ophthalmologic screening. Methotrexate and glucocorticoids remain the most frequent choice for immunosuppressive treatment, though fewer than 7% of patients reported receiving systemic medical therapies. Surgical procedures for cosmetic or functional improvement were common (59%) among the reported patients. Autologous fat grafting was the most frequently utilized cosmetic treatment (50% of procedures) followed by free flap transfers (24% of procedures). There is ongoing need for standardized evaluation, monitoring, and treatment to prevent morbidity in PRS/ECDS, especially in children. When these patients are managed by rheumatologists, methotrexate, and steroids remain the first-line treatment, but a review of the published literature reflects that this may be a minority. Most PRS/ECDS patients are not evaluated in a multidisciplinary fashion. We propose comprehensive evaluations across subspecialties at the baseline and follow-up levels to monitor disease activity and record extracutaneous manifestations, treatment algorithms, and surgical intervention considerations.

Figure 1. a–d

Spectrum of Parry-Romberg syndrome (PRS)/ en coup de sabre (ECDS) patient findings. (a) ECDS lesion of scalp with ipsilateral abnormal T2 signal on brain magnetic resonance imaging (MRI) in a similar plane as the cutaneous findings. (b) PRS/ECDS overlap with skin, soft-tissue, and bone atrophy. (c) PRS/ECDS overlap with single cutaneous band accompanied by significant intraoral manifestations in the same anatomical plane. (d) PRS/ECDS overlap with predominant soft tissue and bone atrophy. “Written publication consent was obtained from the parents of the patients who participated in this study.”

Figure 2

PRISMA flowchart for rapid scoping review process.

Figure 3

Journal topic-category and relative contribution of patient cases. There is apparent over-representation of surgical journals in terms of both publication number and patient cases. Note that journal topic-category may not reflect the medical specialty of the primary authors.

Figure 4

Proposed clinical evaluation, treatment, and monitoring algorithm.