Morphea patients with mucocutaneous involvement: A cross-sectional study from the Morphea in Adults and Children (MAC) cohort

• Published in: Journal of the American Academy of Dermatology Vol. 85; no. 1; pp. 114 - 120
• Main Authors: Prasad, Smriti, Black, Samantha M., Zhu, Jane L., Sharma, Shivani, Jacobe, Heidi
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.07.2021
• Subjects: Adolescent; Adult; Age Factors; Age of Onset; Balanitis Xerotica Obliterans - etiology; Child; Cross-Sectional Studies; Facial Hemiatrophy - complications; Female; Humans; lichen sclerosus atrophicus; localized scleroderma; Localized Scleroderma Activity Index; Localized Scleroderma Damage Index; Male; Middle Aged; morphea; Mouth Diseases - etiology; Mouth Mucosa; mucocutaneous morphea; Physician Global Assessment of Disease Activity; Physician Global Assessment of Disease Damage; Prospective Studies; Scleroderma, Localized - complications; Scleroderma, Localized - pathology; Severity of Illness Index; Vulvar Lichen Sclerosus - etiology; Young Adult; Localized Scleroderma Damage Index; lichen sclerosus atrophicus; localized scleroderma; Localized Scleroderma Activity Index; morphea; Physician Global Assessment of Disease Activity; IQR; LSA; Physician Global Assessment of Disease Damage; mucocutaneous morphea
• ISSN: 0190-9622; 1097-6787; 1097-6787
• DOI: 10.1016/j.jaad.2020.10.093

Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge. To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort. Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018. A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5 years; P < .001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P = .004). Selection bias and limited number of affected subjects. Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.