CPC06 rare case of cutaneous collagenous vasculopathy

Published Date: 19th July 2016

Publication Authors: Ellison J

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We report the case of a 76-year-old woman who presented with a 2-year history of a slowly extending, asymptomatic rash. It had started off on her anterior knees, extending to her arms and trunk. She reported prominence of her rash over the summer months and felt systemically well. Medically she suffered from hypertension, recurrent urinary tract infections, chronic kidney disease stage 3 and gastritis. Her regular medications were doxazosin, simvastatin, irbesartan, bendroflumethiazide, omeprazole, nitrofurantoin and/or cephalexin. On clinical examination she had blanchable telangiectatic macules symmetrically distributed on the limbs and trunk. Darier sign was negative. Initial routine blood tests were essentially unremarkable. Histology revealed vascular ectasia with slight thickening of the vessel wall with hyaline-like material. There was also evidence of solar elastosis, and mast cells were present but not increased in number. The histopathological features were consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). CCV is a rare microangiopathy of the superficial dermal blood vessels. It was first reported in 2000 and is seen most commonly in men between the ages of 50 and 70 years (Davis TL, Mandal RV, Bevona C et al. Collagenous vasculopathy: a report of three cases. J Cutan Pathol 2008; 35: 967-70). CCV often presents with symmetrically distributed blanchable telangiectatic macules usually starting off on the legs, spreading to the trunk and arms. Very rarely it can involve the conjunctiva and mucosa. The rash is usually asymptomatic but can be pruritic. It may be clinically indistinguishable from generalized essential telangiectasia and it has been reported in patients with diabetes mellitus, hypertension, hypothyroidism and psoriasis (Davis et al.). The diagnosis was confirmed on skin biopsy. Histological findings include dilated superficial dermal blood vessels, flat endothelial cells and thickened walls containing hyaline material. The hyaline material stains with periodic acid-Schiff and is diastase resistant. It can also be identified with either Masson's trichrome or immunohistochemistry for laminin and type IV collagen. In most reported cases of CCV therapeutic options are not mentioned. To date there is only one reported case of CCV successfully treated with pulsed dye laser. The prognosis of this rare condition is unclear, and concerns whether CCV may be a marker for connective tissue disease or vascular disease have been raised. Certainly taking biopsies from more cases of clinically suspected essential telangiectasia is advised as this may identify more cases of CCV and lead to better understanding of this very rare condition.

Meah, N; Ellison, J; Khirwadkar, N. (2014).  CPC06 rare case of cutaneous collagenous vasculopathy . British Journal of Dermatology. 171 (Supplement S1), 14

Meah, N; Khirwadkar, N; Ellison, J. (2016). A rare case of cutaneous collagenous vasculopathy . Australasian Journal of Dermatology,. 57 (3), e97-e99.

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