gms | German Medical Science

Introduction: Localized scleroderma in childhood(locSSc) occurs with a prevalence of 3.2 to 3.6 per 10,000 children [1]. There are not many publications assessing in detail the extracutaneous manifestations (EM) of locSSc. It is very important to assess the EM too, because the EM can lead to significant damage and morbidity too.

Methods: Retrospective chart review of all consecutive patients, who were followed at our centre from January 2000 to July 2020 with the diagnosis of locSSc. The subtype was classified according Laxer, et al. [2]. The patients were under the age of 18 years at the time point of the first visit. Demographic and clinical data were extracted.

Results: 73 patients could be identified, 71% of them were female. Mean age at disease onset was 8 years (4–14 years). The mean time of follow up was 5 years. The subtype distribution was 42 (57%) linear, 24 (33%) mixed, 6 (8%) circumscribed morphea and 1(1%) pansclerotic morphea. 9 (21%) of the 42 patients with linear subtype had coup de sabre and 4 (10%) of them had Parry Romberg. Fifty six (76%) patients had EM, 40 (53%) of them had 1 form of EM, 10 (13%) of them 2 forms of EM and 6 (8%) patients 3 forms of EM. 53(73%) of the 76 patients had arthritis. Twenty (37%) of the 53 arthritis involvement occurred on a localisation without overlaying skin involvement. Most frequent localisation of arthritis without overlaying skin involvement was in the hip joints (18%). Of the 53 patients with articular involvement had 31 (58%) linear, 17 (32%) mixed, 4 (7.5) circumscribed morphea and 1 (2%) pansclerotic subtype. 14 (19%) of the 73 had length discrepancy of the extremities and 13 (93%) of them had linear subtype. Neurologic symptoms presenting as headache occurred in 8 (11%) patients, 6 (75%) of them had Parry Romberg subtype and 2 (25%) of them coup de sabre. “White” anterior uveitis was screened according to published recommendations [3], [4] and it occurred in 3 patients, only one of them had coup the sabre the other two linear and mixed subtype without involvement of the face.

Conclusion: EM is very common and it occurs in 76% of the patients. Thirty seven percent of the articular involvement occurred in joints without overlaying skin involvement, which suggest the importance of the whole body joint count as in juvenile idiopathic arthritis. Only 1 of 3 patients with uveitis had skin involvement in the face, which emphasize the recommended uveitis screening.