Cutaneous collagenous vasculopathy (CCV) is a very rare superficial cutaneous microangiopathy of unknown etiology. The disease may have a slight preponderance for females and typically affects middle-aged individuals, although one pediatric case has been reported. Fewer than 50 cases have been documented since it was first described in 2000, but it is probably underdiagnosed, since most clinicians do not routinely perform biopsies of acquired telangiectasias.
Individuals present with diffuse telangiectasias, initially on the lower extremities, that progressively spread to the trunk and upper extremities. Lesions typically spare the head and neck, but involvement of these areas has been observed. Telangiectasias are usually asymptomatic, but associated pruritus or pain has been reported. They also characteristically spare the mucosa and nails, but a 2018 case documented recurrent bleeding from gastric mucosal telangiectasias requiring multiple transfusions.
Cutaneous collagenous vasculopathy
Alerts and Notices
Synopsis

Codes
ICD10CM:
I73.89 – Other specified peripheral vascular diseases
SNOMEDCT:
718634003 – Cutaneous collagenous vasculopathy
I73.89 – Other specified peripheral vascular diseases
SNOMEDCT:
718634003 – Cutaneous collagenous vasculopathy
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Generalized essential telangiectasia (GET) – Clinically indistinguishable. Histology shows dilated capillaries or postcapillary venules in the superficial dermis without thickened walls.
- Hereditary hemorrhagic telangiectasia (HHT) – Systemic involvement, family history, and early onset.
- Hereditary benign telangiectasia – Family history and early onset.
- Telangiectasia macularis eruptiva perstans
- Ataxia-telangiectasia syndrome
- Angioma serpiginosum
- Drug-induced telangiectasias – Calcium channel blockers and interferon alpha most commonly. Histology shows dilated superficial capillaries without thickening of the vessel walls.
- Radiodermatitis
- Collagen vascular diseases (eg, scleroderma, dermatomyositis, lupus erythematosus)
- Primary amyloidosis
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:02/26/2018
Last Updated:04/17/2018
Last Updated:04/17/2018