Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study

Abstract

This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.

Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland

“…Childhood scleroderma affected girls more than boys, consistent with the findings of large retrospective studies (7,8). The mean age at onset was 8.3 years for localized disease and 11.3 years for SSc, compared with 7.3 years (7) and 8.1 years (8), respectively, in previous studies.…”

“…A concern is the delay between the onset of symptoms/signs and being first seen in secondary care: 13 months for localized disease and 7 months for SSc. These compare with intervals between symptom onset and diagnoses, in large retrospective studies, of 19 months for localized disease (7) and 23 months for SSc (8). The median interval between onset of symptoms and diagnosis in the 111 cases of SSc reported by Scalapino et al was 2.8 years (11).…”

“…Herrick et al ciated with the highest morbidity, and is also the subtype most commonly encountered in childhood (7,14). A leaflet describing childhood scleroderma and its different subtypes was sent to all pediatricians along with the first orange card that included childhood scleroderma (July 2005).…”

“…This leaflet included a description of the 2 different subtypes of SSc, as defined on the basis of the extent of skin involvement: limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) (15). Following the initial notification, 2 postal questionnaires were sent to all referring clinicians, including a modified Pediatric Rheumatology European Society (PRES) form (7,8). Clinicians completed the PRES form for either localized disease or SSc, as appropriate.…”

Diagnosis and treatment of systemic and localized scleroderma

“…Eosinophilia occurs in 7-10% of patients with linear or generalized morphea and in a higher percentage of patients with deep morphea [56,57]. Hypergammaglobulinemia is seen in patients with more severe skin disease and is more common during clinical progression.…”