Background/Purpose: Morphea is an autoimmune skin condition that produces skin and soft tissue sclerosis. While clinical manifestations of morphea have been well-described, mucocutaneous findings such as oral and genital lesions have not been well-characterized. Thus, the aim of our study was to determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions.

Methods: The present study was a cross-sectional study of 735 patients in the Morphea of Adults and Children Cohort (MAC Cohort) from 2007 to 2018. We included participants with sufficient demographic and clinical data for analysis. Patient disease activity and damage were assessed by the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), including the Localized Scleroderma Activity Index (LoSAI) and Damage Index (LoSDI). Physician Global Assessment of Disease Activity (PGA-A) and Damage (PGA-D) were recorded. Presence of extragenital and genital lichen sclerosus atrophicus (LsA) and oral mucosa changes were determined by physical exam, histology, and/or referral to relevant specialties. Differences between demographic and clinical variables of groups of interest were analyzed by chi-squared tests for dichotomous variables while Kruskall-Wallis and Mann-Whitney U tests were used for continuous variables.

Results: Of 735 morphea patients, linear comprised 49% (n=362) and generalized comprised 32% (n=232) (Table 1). The median age of morphea onset was older in generalized morphea than linear morphea (55 versus 13 years, p< 0.001). Oral lesions were present in 2.4% (n=18) of patients overall, of which the majority (94%) had linear morphea. Genital lesions were present in 27 (3.7%) of patients overall, the majority of which (85%) had generalized morphea (Table 2). Patients with oral morphea lesions had a younger age of onset than those with genital involvement (11.5 versus 57 years, respectively; p< 0.001). Patients with oral involvement were majority female (78%) while genital involvement were all female and majority Caucasian (89%). Genital morphea patients had LsA overlap in 82% (n=22) versus 0% with oral involvement (p < 0.001). Deep morphea was seen in 100% patients with oral involvement versus 22% of patients with genital involvement (p < 0.001). Median LoSAI and PGA-A scores for patients with oral involvement (0, IQR 0-0 and 0, IQR 0-0, respectively) was lower than patients with genital involvement (6, IQR 0-12 and 13, IQR 0-30, respectively) (p < 0.001 and p< 0.001, respectively). PGA-D scores were higher in oral morphea (32.5, IQR 25.3-48.8) than in genital morphea (15, IQR 10-30) (p =0.001).  

Conclusion: Our study demonstrates mucocutaneous involvement in morphea is uncommon but occurs in a substantial number of those with specific morphea subsets. The presence of mucocutaneous lesions is associated with substantial morbidity. Oral morphea lesions predominate in younger patients with overlying facial linear morphea while genital lesions predominate in post-menopausal women with overlying extragenital LsA. Our results underscore the need to look for mucocutaneous findings in morphea, particularly those with the clinical subsets associated with these findings.

Demographic and Clinical Features of Study Participants (N=735)

Demographic and clinical characteristics by mucocutaneus involvement

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-demographic-features-of-morphea-patients-with-mucocutaneous-involvement-a-cross-sectional-study-from-the-morphea-of-adults-and-children-cohort/