Cutaneous Collagenous Vasculopathy With Ocular Involvement
Practice Points
- Collagenous vasculopathy is an underrecognized entity.
- Although most patients exhibit only cutaneous disease, systemic involvement also should be assessed.
The diagnosis of CCV is confirmed using light microscopy and collagen-specific immunostaining. Examination shows hyaline eosinophilic deposition of type IV collagen around the affected vessels, with the postcapillary venules showing characteristic duplication of the basal lamina.3,9 The material stains positive with periodic acid-Schiff and Masson trichrome.3
Underreporting may contribute to the low incidence of CCV. The clinical presentation of CCV is similar to generalized essential telangiectasia, with biopsy distinguishing the two. Other diagnoses in the differential include hereditary hemorrhagic telangiectasia, which typically would have mucosal involvement; radiating telangiectatic mats and a strong family history; and hereditary benign telangiectasia, which typically presents in younger patients aged 1 year to adolescence.1
Treatment with vascular lasers has been the main focus, using either the 595-nm PDL or the 1064-nm Nd:YAG laser.6,13 Pulsed dye laser or intense pulsed light devices can improve patient well-being1,2; intense pulsed light allows for a larger spot size and may be preferred in patients with a larger body surface area involved.13 However, a few other treatments have been proposed. One case report noted poor response to sclerotherapy.1 In another case, a patient treated with a chemotherapy agent, bortezomib, for their concurrent multiple myeloma showed notable CCV cutaneous improvement. The proposed mechanism for bortezomib improving CCV is through its antiproliferative effect on endothelial cells of the superficial dermal vessels.8 Our patient did not achieve an adequate response with PDL, but the addition of sclerotherapy with polidocanol induced a successful response.
Patients should be examined for evidence of ocular involvement and referred to an ophthalmologist for appropriate care. Although there is no definite association with systemic illnesses or mediation, recent associations with an autoimmune disorder or underlying malignancy have been noted.8,10,11 Age-appropriate cancer screening and attention to associated signs and symptoms are recommended.
