(133) Photodistributed cutaneous collagenous vasculopathy: A diagnostic challenge

Track
Case Reports
Abstract

Cutaneous collagenous vasculopathy (CCV) is a rare cutaneous microangiopathy of unknown etiology affecting the superficial dermal vessels. Clinically, this disorder typically appears as asymptomatic blanchable pink to red macules and telangiectasias that are symmetrically distributed on the bilateral lower extremities. CCV can be progressive with gradual involvement of the upper extremities and trunk. Histopathological features include ectatic and thickened superficial dermal blood vessels with hyalinized deposits lining the basement membrane of affected vessels. Notably there is a lack of perivascular inflammation and the hyalinized vessel walls are PAS-positive and diastase-resistant. Here we describe a case of CCV with unique clinical manifestations including an unusual distribution along with atypical surface change and symptomology. A 54-year-old female presented with a 2-year history of erythematous patches in a photodistribution on the upper chest and symmetric bilateral extensor arms. Scale was present overlying the patches on the arms and chest without any involvement of the lower extremities. The patient reported accompanying severe pruritus and burning pain. A punch biopsy from the arm revealed superficial telangiectatic vessels with PAS-positive hyalinized pink rims. The specimen had an unremarkable epidermis and deep tissue. Considering the histological findings, a diagnosis of CCV was made. This case demonstrates the importance of dermatopathology in identifying CCV, as the clinical presentation can be challenging. The clinical differential for CCV can be broadened to include photodistributed telangiectatic eruptions with accompanying scaly surface changes.

Published in: ASDP 61st Annual Meeting

Publisher: The American Society of Dermatopathology
Date of Conference: November 4-10, 2024