A1 Refereed original research article in a scientific journal
Localized scleroderma and related comorbidities: a single centre cohort study
Authors: Kortelainen, Saara; Hieta, Niina; Rissanen, Tiia; Paltta, Johanna; Pirilä, Laura; Kähäri, Veli-Matti
Publisher: Oxford University Press (OUP)
Publication year: 2025
Journal:British Journal of Dermatology
Article number: ljaf419
ISSN: 0007-0963
eISSN: 1365-2133
DOI: https://doi.org/10.1093/bjd/ljaf419
Web address : https://doi.org/10.1093/bjd/ljaf419
Self-archived copy’s web address: https://research.utu.fi/converis/portal/detail/Publication/505045950
Background: Localized scleroderma, or morphea is a rare autoimmune disease that affects the skin and underlying tissue. It is more common in females than males. The incidence has two peaks, one in childhood and another in middle-age. Concomitant autoimmune diseases are frequently observed, whereas systemic sclerosis occurs rarely simultaneously.
Objectives: This study aimed to assess the clinical features, comorbidities, and treatments of localized scleroderma in Southwest Finland.
Methods: Patients diagnosed with localized scleroderma (ICD-10 code L94) treated between January 1, 2005 and November 30, 2020, were identified from the hospital discharge register of Turku University Hospital. Diagnoses were classified into five main types and their subtypes based on the European Dermatology Forum (EDF) criteria. Basic demographic data, associated comorbidities, treatments used, and their efficacy were collected.
Results: A total of 155 patients with morphea were included, with 125 females (80.6%) and 30 males (19.4%). The most common subtype was limited, plaque-type morphea (n=71, 45.8% of all patients), followed by the generalized type (n=57, 36.8%). Fifty-nine concomitant autoimmune diseases were identified in 45 patients (29.0%), most frequently autoimmune thyroid diseases (n=23, 14.8%). Simultaneous systemic sclerosis was rare (n=3, 1.9%). The most common malignancy was breast cancer (n=11, 7.1%). Extracutaneous manifestations were more common in pediatric-onset patients (18.5%) than in adult-onset patients (1.7%). The most commonly used systemic treatment was methotrexate (n=25, 16.1%) which was beneficial for 64% of treated patients. Phototherapy was administered to 63 patients (40.6%) and it was beneficial for 49 patients (77.8%).
Conclusions: Patients with morphea at our centre often required systemic immunomodulatory treatment or phototherapy. The incidence of the generalized subtype and the occurrence of concomitant autoimmune diseases, particularly thyroid autoimmune diseases, were relatively high. No evidence of an increased risk of malignancy was observed among these patients.
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Funding information in the publication:
This work was supported by Turku University Hospital VTR grants (project no. 30096) awarded to S.K., and (project no. 13336) awarded to V-M.K., as well as by the Research Council of Finland (award no. 363211) and Sigrid Jusélius Foundation, both awarded to V-M.K. The study sponsors had no role in study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the paper for publication. SK is doctoral candidate in the Doctoral Program for Clinical Investigation of the University of Turku.