Cutaneous Collagenous Vasculopathy

Cutaneous Collagenous Vasculopathy

Article
Vein & Vascular Health
Heart & Vascular Health
+3
Contributed byLester Fahrner, MD+1 moreDec 03, 2021

What are the other Names for this Condition? (Also known as/Synonyms)

  • CCV (Cutaneous Collagenous Vasculopathy)
  • Cutaneus Colagenous Vasculopathy

What is Cutaneous Collagenous Vasculopathy? (Definition/Background Information)

  • Cutaneous Collagenous Vasculopathy (CCV) is an extremely uncommon skin condition that is characterized by symmetrical telangiectasias (dilated small blood vessels on skin) appearing as red skin patches, typically on the lower limbs
  • The cause of the condition is not well-understood, but it is believed to be associated with collagen abnormalities in skin. In many cases, Cutaneous Collagenous Vasculopathy may be misdiagnosed as pigmented purpuric dermatosis or generalized essential telangiectasia
  • A treatment of Cutaneous Collagenous Vasculopathy may involve sclerotherapy (injection of medicines into blood vessels) and laser therapy. In many individuals, the prognosis of CCV is generally favorable, since it is not known to cause any severe complications

Who gets Cutaneous Collagenous Vasculopathy? (Age and Sex Distribution)

  • Cutaneous Collagenous Vasculopathy is a rare disorder; fewer than 20 cases have been presently reported in the scientific literature
  • The presentation of symptoms typically occurs in adulthood (age range between 16-83 years)
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Cutaneous Collagenous Vasculopathy? (Predisposing Factors)

Currently, no risk factors have been clearly identified for Cutaneous Collagenous Vasculopathy.

  • The condition is seen to be associated with hypertension and cardiovascular disease in many individuals. However, the cause-effect relationship between CCV and these conditions is unknown
  • Rarely, it is observed in the background of diabetes

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cutaneous Collagenous Vasculopathy? (Etiology)

Currently, the exact cause of Cutaneous Collagenous Vasculopathy is not well-established.

  • Collagen abnormalities in the blood vessels of skin are believed to contribute to the condition
  • Some experts believe that the cause may be related to genetic factors or trauma

What are the Signs and Symptoms of Cutaneous Collagenous Vasculopathy?

The signs and symptoms of Cutaneous Collagenous Vasculopathy may vary among the affected individuals in type and severity. These may include:

  • Skin of reddish discoloration; sometimes, extensive areas of skin may present redness
  • Appearance of prominent blood vessels on skin surface with symmetrical presentation
  • Most of the skin patches are noted on the lower limbs
  • Petechiae (red-purple spots on skin)
  • Easy bruising
  • Presence of skin lesions (macules)
  • Itchiness

How is Cutaneous Collagenous Vasculopathy Diagnosed?

Cutaneous Collagenous Vasculopathy (CCV) is diagnosed on the basis of the following information. A diagnosis of CCV is reportedly very challenging.

  • Physical examination of the individual and medical history evaluation
  • Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin biopsy, if necessary: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • Diagnostic tests to check for an underlying disorder or condition if present

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms. This may include:

  • Generalized essential telangiectasia (GET)
  • Hereditary benign telangiectasia (HBT)
  • Hereditary hemorrhagic telangiectasia (HHT)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cutaneous Collagenous Vasculopathy?

The complications of Cutaneous Collagenous Vasculopathy may include:

  • Bleeding from lesions
  • Scarring of skin due to scratching
  • Cosmetic concerns due to ease of bruising and scarring

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Cutaneous Collagenous Vasculopathy Treated?

The treatment administered for Cutaneous Collagenous Vasculopathy is usually to manage the signs and symptoms and any complications that develop. The treatments considered may include:

  • Conventional sclerotherapy - injecting a solution into the affected veins causing it to shrink and disappear
  • Intense pulsed light therapy - use of high-intensity light to improve skin appearance
  • Pulsed dye or other laser therapies may be recommended in some cases

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

How can Cutaneous Collagenous Vasculopathy be Prevented?

The exact cause and risk factors for Cutaneous Collagenous Vasculopathy are not known, and therefore, no methods or guidelines are available at the present time for its prevention.

What is the Prognosis of Cutaneous Collagenous Vasculopathy? (Outcomes/Resolutions)

  • The prognosis of Cutaneous Collagenous Vasculopathy is dependent upon the severity of the signs and symptoms and associated complications, if any
  • However, in many cases, the condition is not known to present any severe complications, unless it is associated with other comorbidities such as cardiovascular disease

Additional and Relevant Useful Information for Cutaneous Collagenous Vasculopathy:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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