543 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 clinical findings of morphea have been described by our group and others, genital and oral lesions have not been well characterized. To address this knowledge gap, a cross-sectional analysis was performed of patients enrolled in The Morphea in Adults and Children Registry from 2007 to 2018. Of 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%), 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 (86%) had generalized morphea. Patients with oral morphea involvement had a younger age of onset compared to genital involvement (12 and 58 years old, respectively; p < 0.001). Genital morphea patients had extra-genital lichen sclerosus et atrophicus (LsA) in 79% (n=22) as compared to 17% (n= 4) with oral involvement (p < 0.001). Morphea profunda, or deep involvement, was seen in 83% (n = 19) patients with oral involvement versus 14% (n=4) of patients with genital involvement (p < 0.001). Median mLoSSI and 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). Our results show that while mucocutaneous lesions are rare in morphea, oral involvement is predominant in patients with facial linear lesions, particularly PRS, while genital lesions predominate in post-menopausal women with overlying extra-genital LsA. Given the function limiting nature of these lesions, practitioners should know clinical signs that put patients at risk mucocutaneous morphea.