Livedoid vasculopathy is a chronic skin condition that causes recurring painful sores, unusual skin discoloration, and white scars mainly on the lower legs.^{1, 2} It happens when small blood vessels in the skin become blocked by blood clots (thrombi), leading to reduced blood flow to the skin.³ This causes skin cells to die and results in sores (necrosis).

People with livedoid vasculopathy go through periods of disease ‘flare-ups’, called relapse. During relapse new sores appear then scar.^{1, 4} These relapses are followed by periods of where no new sores develop and the pain decreases as the existing sores heal. This period is called remission and can be very short or can last for years. Frequency of relapses (how often the relapses occur) varies from person to person and can be brought upon by rapid changes in temperature.⁵

Synonyms: LV; Livedo reticularis with summer ulceration, livedo reticularis with winter ulcerations, PPURPLE (painful purpuric ulcers with reticular pattern of lower extremities), idiopathic atrophic blanche, white atrophy.²

Livedoid vasculopathy has previously been called livedoid vasculitis, livedovasculitis and segmental hyalinizing vasculitis; however, this terminology is inaccurate as livedoid vasculopathy is not a type of vasculitis, which are diseases involving inflammation of the blood vessels.²

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA: 542643 Livedoid vasculopathy

ICD-11: EF50 Livedoid vasculopathy

People with livedoid vasculopathy experience recurring sores (open wounds/ulcers that continually occur) below the knees.^{1, 2} The condition is made up of three phases:

• early phase – clusters of star shaped purple or red skin discolourations, which often forms a broken net-like pattern (livedo racemosa). The affected area can be very painful and have a burning or itching sensation.
• necrosis phase – fluid filled blisters (bullae) develops in the affected area and burst to form small, deep and painful open sores (ulcers) with a thick crust around them. This phase begins a few days after the early phase.
• third phase – the sores begin to heal and often become white scars called atrophie blanche. This can be slow and can occur over months to years.^{3, 5}

It is common to have multiple sores that are in different phases at the same time. There may be periods of time where there are no sores (remission), but the disease can then flare up (relapse) and new sores may appear.

Please speak to your medical team to learn more about the signs and symptoms of livedoid vasculopathy.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

The exact cause of livedoid vasculopathy is unknown. It is thought that the blood clotting process is affected, leading to blood clots (thrombi) forming easily in the blood vessels just underneath the skin.¹ The blood clots block blood flow and stop skin cells from receiving the nutrients they need to survive. Without nutrients, the skin cells die and form sores on the skin. Decreased blood flow to the skin also impairs wound healing and promotes infections, which contributes to the very slow healing of these sores.

Diagnosis of livedoid vasculopathy may be suspected based on the presence of specific symptoms including:¹

• Stinging or burning pain that happens before (preceding) the appearance of broken net-like pattern of purple or red skin discolouration (livedo racemosa)
• Painful open sores (ulceration)
• White scars (atrophie blanche) on the lower legs

Various blood tests may be performed to look for factors associated with blood clotting and to rule out other conditions.^1,4,7 A small sample of the skin may be taken (skin biopsy) to look for small blot clots under the skin.

As part of the diagnostic process, doctors may do a differential diagnosis, where they rule out other conditions that have similar symptoms – this may include ruling out other causes of microvascular occlusion, vasculitis, diabetes, cancer, skin infections, peripheral arterial occlusive disease, chronic venous insufficiency, and polyarteritis nodosa.^1,5

After a diagnosis of livedoid vasculopathy is confirmed, further tests may be needed to determine if there are any underlying conditions that may increase the likelihood of blood clotting. These can occasionally occur at the same time as livedoid vasculopathy. These include autoimmune diseases such as systemic lupus erythematosus (SLE), clotting disorders (thrombophilias/ hypercoagulable states), chronic venous insufficiency, scleroderma, and neoplasms.^2,6,7

Please speak to your medical team to learn more about the available diagnostic pathways for livedoid vasculopathy.

There is currently no curative treatment for livedoid vasculopathy. Treatment focuses on managing symptoms including pain, preventing recurring episodes, and limiting the severity of the disease.^1,5 These may involve a multidisciplinary medical team. Treatment typically involves:^1,2,5

• Wound care (such as special dressings and medicated creams) can keep the ulcers clean, protect the sores, and speed up the healing process. In some cases, surgical removal of dead skin may also be necessary.
• Pain management  as recommended by a medical professional
• Blood thinners and biological medication to help improve blood flow to an affected area and prevent blood clot formation. These may also be recommended in the absence of a livedoid vasculopathy flare up to reduce the likelihood of recurrence.
• Antibiotics – may be recommended if the ulcers or skin become infected to remove the infection
• Lifestyle changes – it is recommended that people with livedoid vasculopathy avoid large changes in temperatures whenever possible, as large changes in temperature can affect blood flow. Smoking may also be discouraged as it can slow wound healing.

As worsening pain often comes before the development of painful open sores (ulcers), it may also be recommended that individuals with livedoid vasculopathy keep a pain diary.¹ This diary can be useful to predict worsening of the disease and assist doctors in managing treatment. Immediate treatment of the early phase skin discolouration (livedo racemosa) and increasing pain can sometimes prevent progression to the necrosis phase of the disease.

If livedoid vasculopathy-like sores are caused by an underlying condition, in most cases the sores can be treated by treating the causal condition.

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of livedoid vasculopathy may include general practitioners (GP), dermatologists, wound care nurses, rheumatologists, and haematologists.² The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

We are not aware of any clinical care guidelines for livedoid vasculopathy in Australia. If you know of any relevant care guidelines, please let us know via the Contribute page.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• German S1 guideline: diagnosis and treatment of livedovasculopathy; developed by a group of five expert medical societies from Germany, Switzerland, and Austria and published in 2021

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with livedoid vasculopathy at emergency departments/services:

Individuals with livedoid vasculopathy may have a pain diary that can be helpful for healthcare professionals managing treatment. Immediate treatment of the early phase skin discolouration (livedo racemosa) and increasing pain can sometimes prevent progression to the necrosis phase of the disease.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

We are not aware of any rare disease organisations for livedoid vasculopathy in Australia and internationally. If you are aware of any relevant Australian organisations, please let us know via the Contribute page.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Livedoid vasculopathy varies between individuals, and each person’s experience is unique.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on livedoid vasculopathy can be found at:

• National Organization for Rare Disorders (NORD): Livedoid Vasculopathy
• Genetic and Rare Diseases (GARD) Information Center: Livedoid vasculopathy
• Vascular Disease Patient Information Page: Livedoid vasculopathy

Australian Standards for Wound Prevention and Management

Orphanet: Livedoid vasculopathy

Human Phenotype Ontology (HPO): Livedoid vasculopathy

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.