AB012. Clinical and demographic features of morphea patients with mucocutaneous involvement: a cross sectional study from the morphea of adults and children (MAC cohort)

Abstract

While many clinical findings of morphea have been described by our group and others, mucocutaneous findings, including genital or oral lesions, have not been well characterized. To date, we don’t know the frequency, demographic and clinical features associated with mucocutaneus lesions. To address this knowledge gap, we performed a cross-sectional analysis of patients enrolled in the Morphea in Adults and Children Registry from 2007 to 2018. Of the 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), out of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (24/28, 86%) had generalized morphea. Patients with oral involvement had a younger age of onset when compared to patients with genital involvement (12 and 58 years old, respectively; P<0.001). Seventy-nine percent (n=22) of morphea patients with genital involvement had lichen sclerosus et atrophicus (LsA) overlap, compared to 17% (n=4) with oral involvement (P<0.001). Eighty-three percent (n=19) patients with oral involvement were classified as having morphea profunda, or deep involvement, compared to 14% (n=4) of patients with genital involvement (P<0.001). Median mLoSSI ad PGA-A scores for patients with oral involvement (0, IQR 0–4 and 0, IQR 0–24, respectively)) was lower than patients with genital involvement (10, IQR 6–27 and 23, IQR 15–40, respectively) (P<0.001 and P=0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30–60) than in patients with genital involvement (20, IQR 10–25) (P<0.001). In conclusion, have demonstrated that mucocutaneous involvement of morphea lesions occurs most frequently in patients with linear and generalized morphea. Providers may not be aware of the prevalence of these findings and thus they may go undiagnosed for many months or years, leading to the development of potentially permanent and devastating sequalae.