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.
e21555 Background: To examine trends in distant stage melanoma incidence among U.S. women. Methods: Demographic and clinical data were obtained from the United States Cancer Statistics (USCS) Database from 2001-2019. SEER*Stat 8.3.9.2 and Joinpoint regression program 4.9.0.0 were used to calculate cancer incidences and trends per 100,000. Average annual percentage change (AAPC) was used to describe trends. Age-adjusted incidences were adjusted by the U.S. 2000 standard population. Results: From 2001-2019, 543,051 women were diagnosed with distant stage melanoma. Over this period, incidence rose from 0.37 to 0.59/100,000 with an increasing annual rate of 2.58% per year (p < 0.0001). In 2019, non-Hispanic White women had disproportionately higher incidence rates at 0.76/100,000 compared to Hispanic (0.26/100,000), non-Hispanic Black (0.17/100,000), and non-Hispanic Asian (0.08/100,000) women. Regarding race, the largest increase in distant melanoma incidence was observed in White women with an average annual increase of 2.92% (p < 0.0001). Among age groups, incidence was highest among those aged ≥45 (1.54/100,000) and 40-44 (0.53/100,000) years. However, the largest increases in distant melanoma incidence were found among 30-34-year-olds at a rate of 3.91% per year (p = 0.004) and 40-44-year-olds at 2.78% per year (p = 0.003). When examined by race and age, annual incidence rates were highest among White women aged 40-44 years at 3.68% per year (p < 0.0001). Regarding U.S. state, incidence was highest in Florida (1.34/100,000), West Virginia (1.32/100,000), and Oklahoma (1.20/100,000). The largest annual increases in distant melanoma incidence rates were observed in Nebraska at 8.63% (p = 0.001), Kentucky at 6.18% (p < 0.0001), and West Virginia at 6.06% (p = 0.001). When performing an intersection analysis of race, age, and U.S. stage, White women living in Nebraska aged ≥45 had the largest increase in distant melanoma at a rate of 8.12% annually (p = 0.004). In 2019, White women in Utah had the highest incidence of distant melanoma at 3.45/100,000. Conclusions: Distant stage melanoma is disproportionally increasing among adult women aged 30-44 years, especially among White populations. Further studies are warranted to understand the environmental and socioeconomic implications behind these trends.
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