Generalized Morphea: Autoimmune Patterns, Association With Lichen Sclerosus, And Treatment Regimens
Date of Award
Medical Doctor (MD)
Goals and Aims: Generalized morphea is a rare fibrosing disorder marked by collagen deposits in the dermis, causing functional and cosmetic impairment, disability, and poor quality of life. The purpose of our study was to review a cohort of patients with generalized morphea to describe associated autoimmune conditions, prevalence of lichen sclerosus, and treatment modalities.
Methods: We identified patients with a diagnosis of morphea who were examined at Yale Dermatology clinics from 2012 to 2017. An electronic chart review was performed to collect demographic, clinical, and laboratory characteristics.
Results: A total of 60 charts were reviewed of patients seen at our clinics in the last 6 years. There was a female predominance with 82% female patients (N = 49) and 18% male patients (N =11). The racial and ethnic distribution was 77% White (N = 46), 7% African American (N=4), 2% Asian (N=1), and 15% other (Pacific Islander, Native American, multiracial, etc) (N=9). The mean age was 45.8 years (SD = 16.1). The mean interval between generalized morphea onset symptoms and diagnosis was 22.1 months (SD: 17.8). Concomitant autoimmune conditions included vitiligo, systemic lupus erythematosus, Hashimoto’s thyroiditis and alopecia areata. Antinuclear antibodies (ANA) were tested in 72% (43 of 60) of patients and was positive in 58% (25 of 43) of those tested. Extracutaneous manifestations included arthralgia, synovitis, contracture, and carpal tunnel syndrome. Forty-percent of this cohort presented with lichen sclerosus. Eighty percent (16 of 40) had genital lichen sclerosus and 20% (8 of 40) had extragenital lichen sclerosus. Systemic therapies were used most frequently, including methotrexate 87% (52 of 60) and systemic corticosteroids 63% (38 of 60).
Conclusion: In our study, 48% of adult patients with generalized morphea had concomitant autoimmune conditions, and 58% had a positive ANA. This rate is comparable with previous studies and may represent a characteristic systemic pathogenesis. Extracutaneous manifestations of generalized morphea included musculoskeletal symptoms including arthralgia, synovitis, and contractures highlighting the need for multidisciplinary evaluation by rheumatology, orthopedics, and physical therapy. Patients with generalized morphea also have a high prevalence of concomitant lichen sclerosus; a complete evaluation should include a clinical exam of the anogenital region. Lastly, the therapeutic regimens for generalized morphea patients included first-line systemic therapies like methotrexate and corticosteroids, and novel therapies like extracorporeal photochemotherapy.
Castillo Valladares, Herbert B., "Generalized Morphea: Autoimmune Patterns, Association With Lichen Sclerosus, And Treatment Regimens" (2019). Yale Medicine Thesis Digital Library. 3479.
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