Comorbidity of localized scleroderma and primary biliary cholangitis.

BACKGROUND AND OBJECTIVES: Morphea is frequently associated with other autoimmune disorders. Little is known about the association of morphea and primary biliary cholangitis (PBC). The objective of this case series was to study the possible association of morphea with PBC and to identify risk factors. PATIENTS AND METHODS: Patients with morphea were screened for anti-mitochondrial antibodies (AMA) by indirect immunofluorescence and/or immunoblot. Human leucocyte antigen (HLA) genotyping and deep sequencing for the HLA DRB1 subgroup were confirmed in AMA-positive patients. RESULTS: 6 of 91 patients (6.6 %) showed positivity for AMA, and 4 (4.39 %) had PBC. The mean age of AMA-positive patients was 69.0 years. Of 6 AMA-positive patients, common predisposing alleles (HLA DRB1*15: 01 and HLA DRB1*08) were detectable in two patients. One patient had predisposing alleles for both diseases (HLA DRB1*03: 01 and HLA DRB1*14). One patient had a PBC-conferring allele. Female gender, menopause and tendency for remission of morphea were common in all patients. CONCLUSION: The coexistence of morphea, AMA positivity and PBC is a rare but possible association. Common predisposing HLA alleles might interact in such a simultaneous manifestation. We suggest AMA screening for female patients with generalized morphea before the initiation of methotrexate.

Diagnostic and Treatment Strategies of Dermatologists for Treating Morphea in Hungary.

Localized scleroderma is an uncommon disease, only infrequently encountered by dermatologists in private practices or even in larger academic centers. Because of its rarity, current treatment guidelines are mostly based on low-level clinical evidence and expert opinions. The aim of this study was to evaluate treatment strategies to treat localized scleroderma. A questionnaire was developed and sent to dermatologists in Hungary. 101 returned questionnaires were eligible for evaluation. 87.12% of clinicians employed local steroids. Antibiotics were the most preferred systemic agents. Penicillin was used by 32.67% and doxycycline by 22.77% of dermatologists. Methotrexate was employed by only 6.93%. Borrelia serology was obtained by 80.19% of clinicians. More than half of practitioners performed extractable nuclear antigen (ENA) screening (53.46%). Most Hungarian dermatologists did not follow current treatment recommendations for morphea, a trend that likely holds true for other dermatology practices in the East-Central European region as well. Easily accessible, evidence-based guidelines are needed to improve patient care. Patients with localized scleroderma should be referred to specialized centers with more experience where high quality care can be ensured.

Cyclosporine Reduces Sclerosis in Morphea: A Retrospective Study in 12 Patients and a Literature Review.

BACKGROUND: The treatment of severe morphea is challenging, and treatment experience concerning the use of immunosuppressive agents for this condition is limited. OBJECTIVE: The purpose of this study is to analyze the use of cyclosporine, its tolerability, and its effect on skin sclerosis. MATERIALS AND METHODS: Patients with severe morphea who underwent treatment with cyclosporine were studied retrospectively. RESULTS: Five of 12 patients with morphea showed complete remission and 6 patients had partial remission at the end of therapy (9-46 months, median 14) under a median cyclosporine dose of 2.4 mg/kg. The mean affected body surface area fell from 50% (2-80, median 65) to 17% (0-40, median 18). Side effects were hypertension, elevated transaminases, cholesterol, and weight gain. CONCLUSION: Cyclosporine can be effective in morphea. The side effects were reversible. However, the duration of treatment with cyclosporine is limited because of its potential permanent side effects. Prospective placebo-controlled studies are needed to establish the superiority of cyclosporine over other immunosuppressive drugs in this setting.