Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Juvenile Localized Scleroderma of the Face: A Neuro-Cutaneous Disease?

Zulian2,  Francesco, Parolin3,  Mattia, Vittadello3,  Fabio, Calderone3,  Milena, Martini3,  Giorgia, Ullman1,  Susanne

Bispebjerg Hospital, Copenhagen, Denmark
University of Padua, Padova, Italy
University of Padua


Neurological involvement has been reported in few case reports of patients with juvenile localized scleroderma of the face (JLS-F). Aim of the study was to systematically investigate frequency, clinical and radiological features of CNS involvement in JLS-F.


A cohort of consecutive patients with JLS-F (including en coup de sabre (ECDS) and Parry Romberg syndrome (PRS)) underwent a comprehensive clinical evaluation, EEG and brain MRI. All radiographic films were analyzed by 2 neuroradiologists who were blinded to the patients' identity and clinical records. Parenchymal lesions were assessed by a standardized protocol (1) and classified by number, laterality, gray or white matter involvement and concordance with the skin lesion.


34 patients with JLS-F entered the study, F:M ratio was 1.4:1, mean age at disease onset 8,6 years. Twenty nine patients (85.3%) had linear scleroderma of the face, 5 (14.7%) presented a mixed subtype, 23 patients (67.6%) had ECDS, 11 (32.4%) PRS. CNS involvement was found 21 (61.8%) patients, 11 (32,4%) had neurological symptoms such as chronic headache (5), seizures (3), hemiparesis (1), behavioural abnormalities (1) and cranial nerve palsy (1). Ten patients (29,4) presented MRI abnormalities without symptoms. EEG was abnormal in 5 patients (14,7%), all symptomatic and/or with MRI changes. Cerebral MRI resulted abnormal in 17/34 patients (50%). Twelve patients underwent more than one brain MRI which worsened in 5 patients (41,7%), unchanged in 5 (41,7%) and persisted normal in 2 (16.6%). Eight patients had a single brain lesion, 9 a multiple pattern. In 88,2% the site of skin and neurologic lesions were concordant. White matter involvement was present in 10/17 (58,8%), lesions extended to the grey matter in 7 (41,2%).

Four group of patients have been identified: group 1 patients with neurological symptoms and organic brain lesions (mainly multiple with white and grey matter involvement) concomitant or following the onset of the scleroderma skin lesion (no.7), group 2 (no.10) patients with just organic brain lesions following the skin lesions by 1–18 years, mainly single white matter lesions concordant with the site of skin lesion, group 3 (no. 4) patients with neurological symptoms but no organic brain lesions and group 4 (no.13) patients with no neurological involvement.


The high prevalence of neurological involvement in JLS-F reinforces the hypothesis of a possible pathogenetic link CNS-skin in localized scleroderma and confirms the need for a careful clinical and radiological monitoring of every patient since the disease onset.

1.Benseler, S, et al Primary CNS Vasculitis in Children. Arthritis Rheum 2006; 54: 1291-7.

To cite this abstract, please use the following information:
Zulian, Francesco, Parolin, Mattia, Vittadello, Fabio, Calderone, Milena, Martini, Giorgia, Ullman, Susanne; Juvenile Localized Scleroderma of the Face: A Neuro-Cutaneous Disease? [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1682
DOI: 10.1002/art.29447

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