Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences

Background: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given.

Objective: To identify these features which differentiate aLoS from JLS.

Methods: A review of the literature was carried out using the MEDLINE to identify studies assessing demographics, subtype distribution, extracutaneous manifestations, comorbidities, delay at diagnosis and outcome in JLS and aLoS. Non-English articles, reviews, case reports, treatment trials that did not investigate long-term outcomes and studies with the fused data for children and adults were excluded. The analysed papers were published between June 1986 and December 2016.

Results: Fifty-five studies describing JLS or/and aLoS were included for analysis. Female: male ratio in aLoS was higher than in JLS. Adults presented with plaque and generalized subtype more often than paediatric patients. Linear subtype, musculoskeletal, neurologic and ophthalmologic involvement were more frequent, among children. aLoS was likely to be associated with increased prevalence of lichen sclerosus. There was significant delay in diagnosis in both groups of patients. Recurrences of disease were independent of its onset mode. aLoS patients had more poor quality of life scores than did JLS patients. As only a few studies or no studies assessing specified disease subtypes were identified, this limited the ability to compare the adult and paediatric patients with these subtypes.

Conclusion: Despite more favourable course of aLoS, a thorough understanding of the broad spectrum of domains related to physical, mental, emotional and social functioning of patients seems to be important to the proper management of disease. LoS heterogeneity makes it important to develop universal classification criteria.