Unilateral nevoid telangiectatic syndrome. The role of estrogen and progesterone receptors

Unilateral Nevoid Telangiectasia Syndrome

“…Interestingly, dilated arteriolar and venular vessels can be demonstrated histologically in the corium, while signs of endothelial cell proliferation or sprouting in the sense of neoangiogenesis are missing [8]. A pathogenetic importance of sexual hormones, their metabolic products or second messengers activated by signal transduction is postulated [9, 10]. A metabolically caused change in the concentration of adrenergic receptors could contribute directly or indirectly to capillary dilatation.…”

“…Other authors discuss a proliferation of estrogen receptors on skin-relevant cell types within the affected areas. Additionally, the effect of prostaglandins [9]or certain angiogenesis factors (VEGF, EGF) is taken into consideration [2]. …”

When chest pain reveals a Fabry disease

“…They may appear as plaque-like, macular, reticular, mottled, or annular lesions. The telangiectatic lesions of our patient could have been 'unilateral nevoid telangiectasia' [8]. This disorder is the consequence of an elevated circulating estrogen level that acts on vessels in a predisposed area with an increase of estrogen receptors.…”

A curious case of unilateral spider nevi, cirrhosis and stroke: unilateral nevoid telangiectasia syndrome

“…In terms of hormonal factors, Uhlin and McCarty demonstrated an increase in oestrogen and progesterone receptors in the involved skin using a complex radiolabelling method. 5 Subsequent studies have not duplicated this result, although it should be noted that these studies employed the immunoperoxidase method, which is less sensitive in detecting low levels of these receptors. 3 Hynes and Shenefelt proposed that the unilateral localised increase in oestrogen receptors could be caused by somatic mosaicism.…”