Cutaneous collagenous vasculopathy

Alternate names

CCV

Definition

Cutaneous collagenous vasculopathy (CCV) is a primary microangiopathy confined to the skin, characterized by multiple and widespread telangiectasias.

Epidemiology

It is a rare disorder with less than 20 cases reported in the literature to date.

Cause

• The etiology is unknown but CCV is thought to be associated with collagen abnormalities in the skin microvasculature.
• It can be distinguished from generalized essential telangiectasia by characteristic histopathological features that reveal the presence of thickened vascular walls in CCV.

Signs and symptoms

Most patients present in adulthood with symmetrical telangiectasias appearing on the lower extremities and later progressing to the trunk and upper extremities.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

100% of people have these symptoms

• Diffuse telangiectasia

80%-99% of people have these symptoms

• Bruising susceptibility(Bruise easily)
• Erythema
• Macule(Flat, discolored area of skin)
• Petechiae
• Prominent superficial blood vessels(Prominent superficial vasculature)
• Skin rash

30%-79% of people have these symptoms

• Abnormality of metabolism/homeostasis(Laboratory abnormality)

5%-29% of people have these symptoms

• Pruritus(Itching)

NIH genetic and rare disease info

• NIH info on Cutaneous collagenous vasculopathy

Cutaneous collagenous vasculopathy is a rare disease.

Resources

Frequently asked questions

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