Juvenile localized scleroderma: Is it a benign disease?

Abstract

Abstract Introduction Juvenile localized scleroderma is a polymorphic disease. It is more prevalent in girls and has a significant morbidity. Extra-cutaneous involvement is common, and polyautoimmunity can reach 7%. The clinical characteristics of this disease in Colombian patients are currently unknown. Objective To describe the clinical characteristics, morbidity and outcomes in patients with juvenile localized scleroderma in different pediatric rheumatology clinics in Colombia. Materials and methods A descriptive, retrospective, and multicentre study was conducted on patients with juvenile localized scleroderma with a minimum of 1 year of disease onset, and 6 months of follow-up in 10 pediatric rheumatology clinics. Results The study included 88 patients, with a gender distribution of female 2.1: male 1. Mean age at disease onset was 7.1 years (0–14). Mean disease duration at diagnosis was 16.5 months (1–96). Sub-type distribution was, circumscribed (32.9%), mixed (31.8%), and linear (21.5%), that increased to 55% if linear lesions of the mixed subtype are included), generalized (11.4%), and pan-sclerotic morphea (2.3%). Esthetic compromise was detected in 91%, with growth disturbances in 41%, and joint functional compromise in 32%. Extra-cutaneous involvement occurred in 22.7% and polyautoimmunity in 12.5%. Conclusions Juvenile localized scleroderma is a polymorphic and unpredictable disease. It diagnosed late in most of the cases. Extra-cutaneous involvement suggests that is not a disease limited to skin. An early diagnosis, a dynamic treatment and a close follow-up helps to prevent, and detect, complications arising from the disease.