Cutaneous Collagenous Vasculopathy: Description of Two New Cases in Elderly Women and Review of the Literature

Abstract

Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy with characteristic histological findings. It was described in 2000, and 9 cases have since been described. Two women of 83 and 74 years consulted for long-standing telangiectasias. In case 1, they affected the limbs and trunk and in case 2 were located on the legs. Biopsies of these lesions showed dilated vascular structures whose walls were thickened due to deposition of eosinophilic hyaline material. The affected vessels were located in the superficial dermis in case 1, and in case 2 the reticular dermis was also affected. CCV is a microangiopathy of unknown etiology. Clinically it is indistinguishable from generalized essential telangiectasia and differs in its histology. CCV may be underdiagnosed, and some nonbiopsied cases of generalized essential telangiectasia may really be CCV. We contribute 2 new cases of this entity to help establish its clinical and epidemiological characteristics and make its etiology better known.

Cutaneous Collagenous Vasculopathy: A Rare Form of Microangiopathy Successfully Treated with a Combination of Multiplex Laser and Optimized Pulsed Light with a Review of the Literature

“…CCV has almost exclusively been reported in middle-aged subjects [3], even though 1 pediatric case has been reported [4]. In our patient, cutaneous telangiectasia originally developed on the lower legs and progressed subsequently to the thighs, trunk and the upper extremities in accordance with data reported in the literature [5].…”

“…Sparse lymphocytes scattered around the vessel walls may be found. Electron microscopy studies disclose dilated postcapillary venules with flat endothelial cells and a thickened laminated basement membrane consisting of an amorphous eosinophilic hyaline material [2,3]. By immunohistochemistry, vascular cells of endothelial origin are CD31 and CD34 positive.…”

“…CCV has to be differentiated from a number of other vascular disorders presenting with cutaneous telangiectasias, including benign hereditary telangiectasia, angioma serpiginosum and telangiectasia macularis eruptiva perstans [3]. Generalized essential telangiectasia (GET) [8] is another benign condition that should be differentiated from CCV.…”

Cutaneous collagenous vasculopathy: a rare cause of generalised cutaneous telangiectasia

“…Family history is negative for telangiectasias or bleeding disorders and there is no consistent association with a specific medical disorder 2. Although most cases of CCV described had some associated disease and received various treatments, these associations are probably fortuitous and related to the age of the patients 2 8…”

“…CCV is characterised by progressive development of generalised cutaneous telangiectases with unique but subtle histological features, consisting of dilated blood vessels of the superficial dermal plexus with marked wall thickening due to collagen deposition 6 7. Because it is clinically indistinguishable from generalised essential telangiectasia (GET), differing in its histology, it is possible that some cases clinically suspected to be GET may actually represent CCV 8. We describe a new case of CCV to increase awareness of this still poorly known condition and emphasise the importance of clinicopathological correlation in the differential diagnosis of generalised telangiectasias.…”