Clinical and therapeutic course in head variants of linear morphea in adults: a retrospective review

Abstract

Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. This study describes the clinical course, autoimmune co-morbidities, complications, and treatment of adults with PRS/ECDS at a tertiary referral center. We retrospectively reviewed the records of all 34 adult patients with PRS/ECDS identified through billing code search and seen by dermatologists at our institution between 2015 and 2021. Eight patients (23.5%) had ECDS, 8 (23.5%) had PRS, and 18 (52.9%) had overlap. Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. Sixteen patients (47.1%) had a skin biopsy, and 25 (73.5%) had imaging. Forty-six MRIs were obtained, of which 6 (13.0%) reported intracranial findings and 25 (54.3%) reported disease-related connective tissue damage. Twenty-four patients (70.6%) underwent systemic treatment during their disease course per available clinical records. Seventeen patients (70.8%) had improved or stable disease upon treatment completion, with an average duration of 22.2 months. Ten patients (41.7%) reported recurrence of disease following the treatment course. To address changes to facial contour, 6 patients (17.6%) opted for procedural treatments. One patient (16.7%) experienced morphea reactivation following a filler injection performed off-immunosuppression. Compared to findings in children, our study suggests adults with PRS/ECDS are more likely to have oral and ocular complications but experience less severe neurologic symptoms. While systemic treatments appear beneficial in most adult patients with PRS/ECDS, disease may recur following discontinuation.

Use of 3-Dimensional Stereophotogrammetry to Detect Disease Progression in Craniofacial Morphea

“…In both instances, systemic immunosuppression is critical to abrogate disease activity and avoid significant cosmetic and functional sequelae. Even with appropriate treatment, risk for disease recurrence persists, with rates of 28% to 44% reported in the literature . Unfortunately, determining disease progression in CM remains challenging, as clinical findings of disease activity are often lacking.…”

“…Even with appropriate treatment, risk for disease recurrence persists, with rates of 28% to 44% reported in the literature. 2,3 Unfortunately, determining disease progression in CM remains challenging, as clinical findings of disease activity are often lacking. Existing tools include 2-dimensional (2D) photography, clinical documentation, and clinician and/or patient recall, all of which are limited in their ability to detect subtle evolving facial atrophy.…”