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Immunologic Markers of Systemic Scleroderma in Children
Author(s) -
Błaszczyk Maria,
Jabfołnska Stefania,
SzymańskaJagiełło Wanda,
JarzabekChorzelska Maria,
Chorzelski Tadeusz
Publication year - 1991
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1991.tb00832.x
Subject(s) - medicine , scleroderma (fungus) , systemic scleroderma , systemic disease , antibody , disease , pathology , connective tissue disease , immunology , dermatology , autoimmune disease , inoculation
This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp‐2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl‐70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl‐70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl‐70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the sinificance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.