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

• Livedo Reticularis with Summer Ulcerations
• Livedo Reticularis with Winter Ulcerations
• Segmental Hyalinizing Vasculopathy

What is Livedoid Vasculopathy? (Definition/Background Information)

• Livedoid Vasculopathy is described as a chronic and recurrent vasculopathy (blood vessel disease) causing an absence of blood flow in the small blood vessels (capillaries) of the lower legs. The condition causes painful skin lesions that present scarring in both legs
• Livedoid Vasculopathy may be associated with a variety of conditions including coagulation disorders, genetic disorders such as factor V mutation, and arteriosclerosis. The exact cause of development of the condition is unknown, but an increased incidence is noted among pregnant women and during the summer months

Who gets Livedoid Vasculopathy? (Age and Sex Distribution)

• Livedoid Vasculopathy is a rare disorder of the blood vessels
• The presentation of symptoms is commonly observed in middle-aged adults. However, individuals of all age groups, including children, may be affected
• The condition is more common in women than men
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Livedoid Vasculopathy? (Predisposing Factors)

The risk factors for Livedoid Vasculopathy may include:

• A positive family history, since it can be inherited in a few cases
• The following conditions may predispose an individual to Livedoid Vasculopathy:
  • Antiphospholipid syndrome
  • Arteriosclerosis
  • Elevated and abnormal levels of lipoprotein A
  • Factor V mutation
  • Homocysteinemia
  • Protein C deficiency disorder
  • Systemic lupus erythematosus (SLE)
• Pregnant women
• Summer and winter months may cause an exacerbation of the condition

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 Livedoid Vasculopathy? (Etiology)

Presently, the exact cause of Livedoid Vasculopathy is not well-understood. It is not described as a form of vasculitis, which is the inflammation of blood vessels.

• It may arise from blood vessel wall abnormalities in the lower limbs due to certain underlying conditions
• G20210A gene (prothrombin) mutation has been reported in about 8% of the affected individuals

What are the Signs and Symptoms of Livedoid Vasculopathy?

The signs and symptoms of Livedoid Vasculopathy may include:

• Clotting in the small blood vessels of lower legs; mostly the ankles and upper feet surfaces are involved
• Both the legs are almost always affected
• Red or purple marks on affected skin that progress to form ulcers
• Ulceration of skin; ulcers are noted in 1 in 3 individuals
• Presence of white atrophic scars, termed atrophie blanche
• Pain may be mild to moderate

Additional signs and symptoms of the underlying/contributory conditions, if any present, may be noted.

How is Livedoid Vasculopathy Diagnosed?

Livedoid Vasculopathy is diagnosed on the basis of the following information:

• Physical examination of the individual and medical history evaluation; in most cases, a visual exam of the lower extremities may help the healthcare expert in diagnosing the condition
• Blood tests, analysis of pus, and culture of the fluid being drained, as needed
• 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
• Imaging studies of the affected region
• Skin biopsy, if necessary: A skin tissue 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

Further testing may be needed to diagnose any associated/underlying condition.

A differential diagnosis may be undertaken to exclude other medical conditions that present similar signs and symptoms. These may include:

• Arterial insufficiency
• Arterial ulceration
• Cutaneous polyarteritis nodosa
• Diabetic foot ulcers
• Malignant atrophic papulosis
• Rheumatoid arthritis
• Small vessel vasculitis
• Systemic lupus erythematosus (SLE)
• Systemic sclerosis
• Venous insufficiency

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 Livedoid Vasculopathy?

The complications of Livedoid Vasculopathy may include:

• Severe scarring
• Emotional distress
• Persistence and recurrence of the ulcers
• Complications that may arise from the underlying condition, if any present

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

How is Livedoid Vasculopathy Treated?

There are no well-defined treatment measures available for Livedoid Vasculopathy. The main treatment goals are to control the symptoms and treat the underlying causative condition, if any present. This may involve the following measures:

• Prevent further aggravation of the skin lesions (including cessation of smoking)
• Wound care and dressing
• Antibiotic therapy to treat secondary infections, if needed
• Removal of dead material from the ulcers (debridement)
• Elevation of leg while resting or sleeping
• Light therapy via PUVA
• Hyperbaric oxygen therapy

Administration of suitable medications to improve blood flow in the region and prevent blood from clotting. These may include:

• Antiplatelet agents (to reduce risk of blood clots)
• Fibrinolytic and anticoagulant agents (to prevent clotting)
• Vasodilating agents
• Anti-inflammatory medications
• Vitamin supplementation, as needed

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

How can Livedoid Vasculopathy be Prevented?

Livedoid Vasculopathy may not be preventable, if it is a genetic disorder that runs in the family.

• If there is a family history of the condition, then genetic counseling will help assess risks
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
• Undertaking early diagnosis and treatment of an associated/predisposing condition is important

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

• The prognosis of Livedoid Vasculopathy is dependent upon the severity of the signs and symptoms and associated complications, if any
• The condition is chronic and may last anywhere from a few months to even up to 21 years. Once remission occurs, the scars are known to slowly fade over time

Additional and Relevant Useful Information for Livedoid Vasculopathy:

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

http://www.dovemed.com/diseases-conditions/rare-disorders/