Cutaneous Collagenous Vasculopathy: A Retrospective Clinical and Histopathologic Analysis of 34 Cases

Abstract

Background: Cutaneous collagenous vasculopathy (CCV) is a rare acquired microangiopathy that commonly affects middle-aged, fair-skinned individuals. It presents with telangiectatic lesions involving the lower extremities. Histologic analysis is mandatory for diagnosis. Objectives: To describe and characterize the patients diagnosed with CCV at the Mayo Clinic. Methods: A multicenter retrospective observational analysis was performed on patients with a clinical and histological diagnosis of CCV at the Mayo Clinic in Florida, Arizona, and Rochester from January 2000 to October 2023. Results: The study included 34 patients, 22 (64.7%) females. The median age at diagnosis was 52 years (range, 12-80 years). CCV lesions were found to affect the lower extremities in 29 (85.3%) patients, followed by the trunk in 14 (41.2%). Nineteen (55.9%) patients presented 1 concomitant medical condition, 8 (23.5%) 2 conditions, and 5 (14.7%) 3 or more medical conditions, such as arterial hypertension 10 (29.4%), type 2 diabetes mellitus 3 (8.8%), hyperlipidemia 3 (8.8%), cardiac disease 3 (8.8%), and hematologic malignancy 3 (8.8%). Thirty-one patients (91.2%) were taking medications, with 19 (55.9%) taking 3 or more. Pathology staining revealed periodic acid-Schiff positivity in 23 of 34 cases (67.6%), and collagen IV in 26 of 34 cases (47.1%). Treatments included pulsed dye laser 3 (8.8%) and topical sirolimus 1 (2.9%). Conclusions: CCV is an uncommon microangiopathy that affects pediatric, middle-aged, and elderly patients. It involves the lower extremities, trunk, and upper extremities. Patients often present with associated metabolic and cardiovascular conditions and are usually taking at least 1 medication. Treatment options are limited.