Localized scleroderma in childhood is not just a skin disease.
OBJECTIVE
Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma.


METHODS
Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement.


RESULTS
Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased.


CONCLUSIONS
Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.
Zulian
Francesco
F
Dipartimento di Pediatria, Università di Padova, Padua, Italy. zulian@pediatria.unipd.it
Vallongo
Cristina
C
Woo
Patricia
P
Russo
Ricardo
R
Ruperto
Nicolino
N
Harper
John
J
Espada
Graciela
G
Corona
Fabrizia
F
Mukamel
Masha
M
Vesely
Richard
R
Musiej-Nowakowska
Elzbieta
E
Chaitow
Jeff
J
Ros
Joan
J
Apaz
Maria T
MT
Gerloni
Valeria
V
Mazur-Zielinska
Henryka
H
Nielsen
Susan
S
Ullman
Susanne
S
Horneff
Gerd
G
Wouters
Carine
C
Martini
Giorgia
G
Cimaz
Rolando
R
Laxer
Ronald
R
Athreya
Balu H
BH
D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D002648:Child; D015331:Cohort Studies; D005128:Eye Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D005767:Gastrointestinal Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D006331:Heart Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D007592:Joint Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D007674:Kidney Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D009422:Nervous System Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D015995:Prevalence; D012140:Respiratory Tract Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology