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)
Diagnosis
Treatment
NIH genetic and rare disease info
Cutaneous collagenous vasculopathy is a rare disease.
Resources
Frequently asked questions
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