Childhood‐onset scleroderma: Is it different from adult‐onset disease?

Abstract

Objective. To distinguish childhood‐onset scleroderma from adult‐onset disease. Methods. The clinical and serologic features of 58 patients with childhood‐onset scleroderma (11 patients with diffuse cutaneous systemic sclerosis [SSc], 16 with linear SSc, 14 with linear morphea, and 17 with morphea) were examined in the largest cohort of such patients studied to date. These parameters were compared with data obtained from patients with adult‐onset disease. Results. Childhood‐onset scleroderma resembled adult‐onset disease with regard to the heterogeneity of clinical expression and subsets of disease, but it also differed from adult‐onset disease in a number of clinical and laboratory parameters. The predominant childhood‐onset disease presentation was the localized form of the disease, with limited and diffuse SSc being less notable. There was a significant association of trauma with childhood‐onset scleroderma (P < 0.0001), which was not noted in adult‐onset disease. Furthermore, in contrast to adult disease, patients with childhood‐onset disease had normal levels of parameters of vascular activation (von Willebrand factor, angiotensin‐converting enzyme, E‐selectin, and endothelin‐1), T cell activation (soluble interleukin‐2 receptors), and collagen synthesis (carboxy‐terminal type I and aminoterminal type III), a notable lack of anticentromere antibodies, and abnormal coagulation indices. Conclusion. A number of features distinguish childhood‐onset scleroderma from adult‐onset disease.

Localized scleroderma in childhood is not just a skin disease

“…This hypothesis is, in part, supported by our finding of RF positivity in a significantly higher number of patients with arthritis and, in general, in those with the linear subtype of SSc. This finding has already been reported in previous studies (10,11,13,14).…”

“…It was more frequent in patients with the linear subtype of juvenile localized scleroderma, in whom the prevalence of this complication was already reported to range from 30% to 52% (10)(11)(12). Although it is conceivable that linear bands of sclerosis, spreading across articular structures, can cause inflammation and joint contractures by a direct local mechanism, it was quite surprising that in one-fourth of these patients, arthritis was completely unrelated to the site of the skin lesion, raising the suspicion of a systemic, rather than a local, inflammatory process.…”

Epidemiologi ved sklerodermi hos barn

“…Sjelden sykdom, men trolig opptrer lokalisert sklerodermi 10 ganger hyppigere enn systemisk sklerose som man antar opptrer hos <1/1.000.000 barn pr. år (1,4). Mens det hos voksne er plaque morfea som er den hyppigste formen er det fra barnerevmatologiske sentere rapportert at lineaer sklerodermi er hyppigst hos barn (1).…”

Childhood‐onset scleroderma from a dermatologist's perspective: Comment on the article by Vancheeswaran et al

“…One aspect not reported in the paper which may have a significant bearing on the estimated incidence of MPA is the clinical and pathologic features of the 27 patients in the Norfolk (UK) registry who were classified as having Wegener's granulomatosis (WG) by the ACR criteria (1). As the authors themselves point out, there can be a significant overlap between WG and MPA, both clinically and with respect to antineutrophil cytoplasmic antibody specificity (1). While upper respiratory tract and otolaryngologic involvement tends to favor a diagnosis of WG, the "nasal or oral inflammation" defined by the ACR criteria is reported in up to 20% of patients with MPA (2).…”