Pathogenesis of telangiectasia in scleroderma.

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dc.contributor.authorMould, T Len_US
dc.contributor.authorRoberts-Thomson, P Jen_US
dc.date.accessioned2009-05-27T17:25:27Z
dc.date.available2009-05-27T17:25:27Z
dc.date.issued2000-12-24en_US
dc.descriptionPublished by the Allergy and Immunology Society of Thailand.en_US
dc.description.abstractScleroderma (progressive systemic sclerosis) is a systemic autoimmune disorder characterised by skin sclerosis, calcinosis and changes in microvasculature. The etiology of the disease is unknown but both genetic and environmental factors have been implicated. Telangiectasia (macroscopically visible dilated skin vessels) occurring primarily on the hands and face, are a prominent feature in scleroderma and are present in the majority of patients. Similarly, telangiectasia are found in patients with hereditary hemorrhagic telangiectasia (HHT), a mutational disorder of the germline genes endoglin and ALK-1, members of the TGFbeta receptor family, expressed on endothelial cells. Our study investigated the number, distribution and microscopic characteristics of telangiectasia in both limited (n = 29) and diffuse scleroderma (n = 9) and compared findings with 3 patients with HHT. In limited scleroderma, the mean number of telangiectasia (hand and face) was 36 (0-150) compared with 23 (0-135) in diffuse scieroderma. A significant correlation was observed between the number of telangiectasia on the face and on the hands (p = 0.014). The total number of telangiectasia correlated significantly with the disease duration (p = 0.009). The spatial distribution of the telangiectasia appeared to be random on both hands and foreface in contrast with the distribution of subcutaneous calcification of the hands which occurred predominantly on the distal and flexor surfaces of the first, second and fifth digits. Nailfold microscopic capillaroscopy was performed on 12 patients. No significant correlation was observed between capillary diameter or density and with total number of telangiectasia observed macroscopically. The distribution and microscopic appearance of telangiectasia in scleroderma appeared very similar to those observed in HHT. In view of these similarities we therefore conclude that telangiectactic development in scleroderma may be associated with disorders of the TGFb receptor family proteins found on the microvasculature.en_US
dc.description.affiliationDepartment of Immunology, Allergy and Arthritis, Flinders Medical Centre, Bedford Park, South Australia.en_US
dc.identifier.citationMould TL, Roberts-Thomson PJ. Pathogenesis of telangiectasia in scleroderma. Asian Pacific Journal of Allergy and Immunology. 2000 Dec; 18(4): 195-200en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/37161
dc.language.isoengen_US
dc.subject.meshAdulten_US
dc.subject.meshAgeden_US
dc.subject.meshAged, 80 and overen_US
dc.subject.meshCapillaries --pathologyen_US
dc.subject.meshFace --blood supplyen_US
dc.subject.meshFemaleen_US
dc.subject.meshHumansen_US
dc.subject.meshMaleen_US
dc.subject.meshMiddle Ageden_US
dc.subject.meshNails --blood supplyen_US
dc.subject.meshScleroderma, Systemic --complicationsen_US
dc.subject.meshSkin --blood supplyen_US
dc.subject.meshTelangiectasis --etiologyen_US
dc.titlePathogenesis of telangiectasia in scleroderma.en_US
dc.typeJournal Articleen_US
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