Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy

Abstract

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. Microscopically, cutaneous collagenous vasculopathy resembles the superficial telangiectasias of generalized essential telangiectasia but additionally shows hyaline material in thickened vessel walls. The amorphous pink material is periodic acid-Schiff-positive and resistant to diastase. We describe a series of four patients with cutaneous collagenous vasculopathy and highlight its clinical and histopathologic features.

Cutaneous collagenous vasculopathy: a rare cause of generalised cutaneous telangiectasia

“…Clinically, it is characterised by the progressive development of symmetric telangiectatic macules that typically first arise in the lower extremities and gradually spread to reach the trunk and upper limbs, sparing the mucosal surface and nail bed 2 9. The head and neck are usually not involved 2.…”

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

“…Our patient had clinical and histopathological features typically described as CCV, a distinctive primary cutaneous microangiopathy first described in 2000 [1,2]. This entity is rare since only 25 cases have been described so far in the English literature (table 1).…”

“…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.…”

“…Usually lesions are almost invariably asymptomatic although some itching may occur [5]. Lesions remain then relatively stable or may occasionally worsen during the summer months [2]. No mucosal involvement has been reported so far.…”

“…At variance with CCV, GET is more common in women and can rarely also involve the oral mucosa and conjunctiva. In contrast to CCV, in GET there are dilated vessel walls, which consist of thickened endothelial cells, but without any deposition of amorphous hyaline material [2,9]. …”