Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre"

L. Chini, A. Orlacchio, R. Silenzi, F. Della Gatta, R. Iannini, E. Monteferrario, S. Spano, M. Guarino, V. Moschese, G. Simonetti

Research output: Contribution to journalArticle

Abstract

Scleroderma is a group of rare chronic connective tissue disorders characterized by collagen accumulation that causes tissue hardening with consequent fibrosis. Skin involvement is mostly frequent, although several internal organs can be impaired (heart, lungs, liver, etc.). In childhood, juvenile localized scleroderma (JLS) is more frequently observed; in this subtype cutaneous lesions pre-dominate frequently on the limbs but also on the face and scalp; in this case, it is referred to as scleroderma "en coup de sabre" (ECDS). Neurological abnormalities have been described in association with ECDS as an effect of progressive scalp and underlying tissues involvement. Up to now, no validated biomarkers exist to evaluate disease evolution and, in this way, frequently diagnosis of central nervous system (CNS) involvement occurs when patients are already symptomatic. We describe the case of a 5-year old girl, with a diagnosis of ECDS characterized by the typical scalp lesion, with slight subsidence of the underlying diploe. In this case, radiological examination has been essential to evaluate the degree of progression of skin and soft tissues lesions and to clarify the right therapeutic approach. In selected JLS children, both MRI and CT with 3D reconstruction images provide a useful tool to monitor disease evolution and to address therapeutic choices.

Original languageEnglish
Pages (from-to)89-93
Number of pages5
JournalMinerva Pediatrica
Volume66
Issue number1
Publication statusPublished - 2014

Fingerprint

Localized Scleroderma
Scalp
Neuroimaging
Skin
Computer-Assisted Image Processing
Connective Tissue
Fibrosis
Collagen
Extremities
Central Nervous System
Biomarkers
Lung
Liver
Therapeutics
Juvenile-onset scleroderma

Keywords

  • Child
  • Scalp
  • Scleroderma, localized

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Chini, L., Orlacchio, A., Silenzi, R., Della Gatta, F., Iannini, R., Monteferrario, E., ... Simonetti, G. (2014). Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre". Minerva Pediatrica, 66(1), 89-93.

Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre". / Chini, L.; Orlacchio, A.; Silenzi, R.; Della Gatta, F.; Iannini, R.; Monteferrario, E.; Spano, S.; Guarino, M.; Moschese, V.; Simonetti, G.

In: Minerva Pediatrica, Vol. 66, No. 1, 2014, p. 89-93.

Research output: Contribution to journalArticle

Chini, L, Orlacchio, A, Silenzi, R, Della Gatta, F, Iannini, R, Monteferrario, E, Spano, S, Guarino, M, Moschese, V & Simonetti, G 2014, 'Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre"', Minerva Pediatrica, vol. 66, no. 1, pp. 89-93.
Chini L, Orlacchio A, Silenzi R, Della Gatta F, Iannini R, Monteferrario E et al. Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre". Minerva Pediatrica. 2014;66(1):89-93.
Chini, L. ; Orlacchio, A. ; Silenzi, R. ; Della Gatta, F. ; Iannini, R. ; Monteferrario, E. ; Spano, S. ; Guarino, M. ; Moschese, V. ; Simonetti, G. / Neuroimaging is useful for monitoring disease activity in linear scleroderma "en coup de sabre". In: Minerva Pediatrica. 2014 ; Vol. 66, No. 1. pp. 89-93.
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