Collagen XVIII and fibronectin involvement in bullous scleroderma.
BACKGROUND
Endostatin, an anti-angiogenic C-terminal fragment of collagen XVIII, has been recently reported to play a role in scleroderma pathogenesis, but collagen XVIII immunohistochemistry in scleroderma skin has still not been performed. Bullous scleroderma, a rare form of scleroderma, may have altered angiogenic and lymphangiogenic characteristics.


OBJECTIVE
Our aim is to report a rare case of bullous scleroderma, studying the presence of fibronectin and collagens type I, III and XVIII in sclerodermic skin.


METHODS
We describe the progression of bullous scleroderma in a 67-year-old patient since the first symptoms. Histological and immunohistochemical aspects of skin biopsies are compared to normal skin from a patient without scleroderma and are correlated with the pathogenesis of the disease. Indirect immunofluorescence measured by laser confocal microscopy allows quantitative determination of fibronectin and collagens type I, III and XVIII.


CONCLUSIONS
Dermo-epidermal cleavage, fibrosis and inflammation are the main histological findings. The dermal distribution and amounts of collagens and in the scleroderma patient are similar to normal skin. Conversely, both fibronectin and collagen XVIII are increased in scleroderma skin, suggesting their involvement in the pathogenesis of bullous scleroderma.
Santos
Soraya Neves Marques Barbosa dos
SN
Hospital Semper, Belo Horizonte, Minas Gerais, Brazil. sorayasantos@doutoradermatologista.com.br
Oliveira
Gisele Viana de
GV
Tavares
André Luiz Pasqua
AL
Massensini
André Ricardo
AR
Carvalhaes
Lorenza Machado de Souza
LM
Reljasvaara
Ritva
R
Kitten
Gregory Thomas
GT
D000368:Aged; D043170:Collagen Type XVIII / Q000378:metabolism*; D005260:Female; D005353:Fibronectins / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D012594:Scleroderma, Localized / Q000378:metabolism / Q000473:pathology*; D012872:Skin Diseases, Vesiculobullous / Q000378:metabolism / Q000473:pathology*
D043170:Collagen Type XVIII; D005353:Fibronectins