Immunologic dysfunction in scleroderma: evidence for increased mast cell releasability and HLA-DR positivity in the dermis.
To investigate the role of mast cells and cell-mediated immunity in the pathogenesis of scleroderma, we studied wheal size after skin testing with compound 48/80, a liberator of mast cell histamine, and demonstrated increased mast cell releasability in skin that appeared normal, adjacent to involved skin. Immunofluorescent staining for HLA-DR showed dermal positivity in 12 of 13 involved- and 9 of 13 uninvolved-skin biopsy specimens from scleroderma patients, compared with only 1 of 10 controls. By immunoperoxidase staining, most of the DR positivity was found in fibroblast-like cells. These findings further support the notion of immunologic dysfunction in scleroderma.
Pearson
M E
ME
Department of Medicine, University of Colorado Health Sciences Center, Denver 80262.
Huff
J C
JC
Giorno
R C
RC
Panicheewa
S
S
Claman
H N
HN
Steigerwald
J C
JC
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005455:Fluorescent Antibody Technique; D006681:HLA-D Antigens / Q000032:analysis*; D006684:HLA-DR Antigens / Q000032:analysis*; D006801:Humans; D007107:Immune System / Q000503:physiopathology*; D007124:Immunoenzyme Techniques; D008407:Mast Cells / Q000502:physiology*; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000473:pathology / Q000503:physiopathology*; D012867:Skin / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D012882:Skin Tests; D013194:Staining and Labeling
D006681:HLA-D Antigens; D006684:HLA-DR Antigens