543 Clinical and demographic features of morphea patients with mucocutaneous involvement: A cross sectional study from The Morphea of Adults and Children (MAC Cohort)

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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.