Cutaneous collagenous vasculopathy: a new case series with clinicopathologic and ultrastructural correlation, literature review, and insight into the pathogenesis.

Samih S Salama
Samih S Salama
St Joseph's Healthcare and McMaster University
Hamilton | Canada

Am J Dermatopathol 2015 May;37(5):368-75

Department of Pathology (Dermatopathology), St Joseph's Healthcare and McMaster University, Hamilton, Ontario, Canada.

Cutaneous collagenous vasculopathy (CCV) is a rare distinct idiopathic microangiopathy of the superficial cutaneous vasculature. Seven new cases are reported (6 females and 1 male) ranging in age from 42 to 85 years, with some showing unusual clinical and histopathological findings. All presented with macular telangiectases starting on the lower extremities and spreading progressively in 5 cases and were suspected to have generalized essential telangiectasia. Two cases had a history of over 20 years. One case had lesions in the abdominal striae, and 1 was markedly ecchymotic. All skin biopsies showed the characteristic features of CCV with dilatation and marked thickening of the walls of superficial dermal blood vessels displaying reduplication of the basement membrane on periodic acid-Schiff-diastase stain and deposition of hyaline collagenous material immunostaining as collagen type IV, and showing decreased or absent actin staining. However, the changes were subtle and only seen focally in some biopsies. Few lymphoid cells were present around occasional vessels. Electron microscopy showed increased basement membrane lamellae with marked deposition of normal and some abnormal collagen (Luse-like bodies) and focal endothelial damage, suggesting reparative perivascular fibrosis resulting from repeated endothelial injury. These cases (and all 18 previously reported ones) are of a wide age range and no gender predilection. This disorder is underdiagnosed, and it is likely that some cases clinically suspected to be generalized essential telangiectasia may actually represent CCV. Better recognition by dermatologists may lead to more biopsies from patients with generalized telangiectasia and a further understanding of the pathogenesis of CCV and its relationship to other cutaneous vascular disorders.
PDF Download - Full Text Link
( Please be advised that this article is hosted on an external website not affiliated with
Source Status
Web SearchFound
Publisher SiteFound ListingPossible
May 2015
6 Reads

Similar Publications

Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature.

Dermatology 2012 19;225(1):1-8. Epub 2012 Jul 19.

Dermatology Service, Central University Hospital of Asturias, Oviedo, Spain.

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. Read More

View Article
March 2013

Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy.

J Cutan Pathol 2012 Aug 16;39(8):741-6. Epub 2012 Jul 16.

Cleveland Clinic - Anatomic Pathology and Dermatology, Cleveland, OH, USA.

Cutaneous collagenous vasculopathy is a rare microangiopathy of superficial dermal blood vessels. Patients present with telangiectatic macules, predominantly on the extremities. A skin biopsy specimen is necessary to distinguish cutaneous collagenous vasculopathy from generalized essential telangiectasia. Read More

View Article
August 2012

Cutaneous collagenous vasculopathy: Report of first Japanese case and review of the literature.

Australas J Dermatol 2017 May 28;58(2):145-149. Epub 2016 Jan 28.

Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.

Cutaneous collagenous vasculopathy (CCV) is a rare acquired idiopathic microangiopathy characterised by the progressive development of diffuse asymptomatic telangiectasias over the skin. Histologically, the presence of a thick hyaline collagenous wall around the affected capillaries, comprising the accumulation of collagen type IV, is noted. We herein report the case of a 17-year-old Japanese boy with symmetrical patches of diffuse telangiectasias on the bilateral extremities that persisted for 10 months. Read More

View Article
May 2017

Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study.

J Cutan Pathol 2000 Jan;27(1):40-8

Department of Pathology, St. Joseph's Hospital and McMaster University, Hamilton, Ontario, Canada.

We report a 54-year-old male, with a 5-year history of spreading asymptomatic generalized cutaneous telangiectases. The patient had no mucosal or nail involvement, no positive family history and no clinical evidence of systemic disease or bleeding diathesis. Histologically, the superficial small dermal blood vessels were dilated and showed thickened walls with hyaline perivascular material, staining as collagen. Read More

View Article
January 2000