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

Abstract

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. The vessel walls were PAS and colloidal iron stain positive, and immuno-histochemically lacked actin staining. Collagen IV, fibronectin and laminin antibodies showed the material deposited around the basement membrane zone. Ultrastructurally, the vessels were post-capillary venules (PCV) and showed marked collagen deposition around the basal lamina. There were many abnormally banded widely spaced fibres with 100-150 nm periodicity (Luse bodies), in addition to regular banded collagen. Pericytes were sparse and lacked intracytoplasmic filaments, and few veil or fibroblastic cells were seen embedded within the collagen. We believe this is a form of cutaneous microangiopathy not previously described, with distinct morphology and unique ultrastructural features. It may be due to a genetic defect with erroneous production of disorganized collagen in the cutaneous microvasculature. Dermatologists and Dermatopathologists should be aware of this unusual cutaneous vasculopathy.

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

“…Electron microscope analysis shows that the affected vessels correspond to postcapillary venules. Around the basal lamina of these venules is deposited a granular material with medium electrodensity within which collagen fibers are irregularly arranged [1,2]. In these fibers transverse bands are observed with anomalous periodicity (Luse bodies) that are not unique to CCV.…”

“…This thickening is due to deposition of eosinophilic material around the endothelial basal lamina showing positivity with PAS staining and is stained blue with Masson trichrome. Mucinous material is often found [1,2,3,4,5,6] in this deposit. It has been reported that collagen IV, fibronectin and laminin are positive [1,2] but in our case No.…”

“…Salama and Rosenthal [1] described CCV in a 54-year-old male with scattered telangiectasias. Histologically, the telangiectasias corresponded to dilated postcapillary venules whose walls were thickened due to deposition of collagen on the vascular walls.…”

“…All reported cases had some associated disease, and most were taking medication on a chronic or intermittent basis [1]. In some cases, however, this is not specified [3].…”

“…Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy first described by Salama and Rosenthal [1] in 2000. The key to diagnosis is finding dilated venules with deposits of eosinophilic hyaline material located in the vessel wall.…”