Abstract
Head variant linear morphea includes en coup de sabre (ECDS), involving the paramedian forehead and Parry Romberg syndrome (PRS), presenting with hemifacial atrophy. Currently, literature describing presentation and clinical course of PRS/ECDS in adults is sparse. We retrospectively reviewed the clinical features and treatment course of 34 adult patients with PRS/ECDS, 28 females (82.4%) and 6 males (17.6%), at the University of California San Francisco Department of Dermatology. Eight patients (23.5%) had ECDS, 8 (23.5%) had PRS, and 18 (52.9%) had overlapping head variants; head involvement was unilateral in all patients. Eleven patients (32.4%) had extrafacial morphea involvement, including localized, generalized, linear morphea of the trunk/extremities, and/or genital lichen sclerosus; bilateral involvement was noted in 6 patients (17.6%). Twenty-one patients underwent systemic treatment, including methotrexate, mycophenolate, corticosteroids, and/or antimalarials. Seventeen patients (81.0%) had improved or stable disease upon completion of treatment, with an average treatment duration of 32.75 months. Five patients (24.0%) reported recurrence of disease after treatment, with time to recurrence ranging from 8 to 120 months. To revise facial disfigurement, 6 patients opted for autologous fat filler, commercial filler, and/or infraorbital implant. One patient experienced morphea reactivation following a cosmetic procedure performed off-immunosuppression. Our study suggests that while systemic immunomodulators are effective in most adult patients with PRS/ECDS, disease recurrence is not uncommon following discontinuation. Further study is needed to clarify optimal timing for fillers and filler agent. Compared with findings in children, our study suggests adults may be more likely to have overlapping variants of head morphea.
Published Version
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