Abstract

A 40-year-old female with a history of Sjogren's syndrome presented with diffuse asymptomatic pink telangiectatic patches on the bilateral lower extremities. Biopsy of the lesion revealed ectatic superficial dermal small blood vessels with thickened vessel walls and scanty inflammation. Periodic acid-Schiff (PAS) staining highlighted the presence of hyaline deposits in these vessel walls. These findings were most consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). CCV is a rare primary cause of generalized telangiectasia but is potentially underrecognized clinically leading to fewer biopsies. This idiopathic, acquired microangiopathy affects primarily the superficial dermal vessels demonstrating marked dilatation and vascular hyaline deposition. PAS stains the hyaline material within vessel walls often revealing a split basement membrane. Clinically, this entity tends to present with macular telangiectasias predominantly on the lower extremities with progression to the trunk and upper extremities but sparing the head, neck and mucous membranes. The lesions are usually asymptomatic but can be pruritic. There is usually no family history, and it is not associated with any specific medical condition or syndrome. Though less than 50 cases of CCV have been reported, a few cases have been documented in patients with a history of connective tissue disease including Sjogren’s syndrome, as in the case of this patient. The potential link between connective tissue disease and CCV warrants further investigation into the role that a heightened inflammatory milieu might have on the pathogenesis of CCV.

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