Livedo reticularis

Author: Vanessa Ngan, Staff Writer, 2003. Updated by Dr Sara de Menezes, Basic Physician Trainee, Alfred Health, Melbourne, Australia; Chief Editor, Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, September 2016.


What is livedo reticularis?

Livedo reticularis refers to various conditions in which there is mottled discolouration of the skin. It is described as being reticular (net-like, lace-like), as cyanotic discolouration surrounds pale central skin. 

The terminology of livedo reticularis may include: 

  • Cutis marmorata: physiological, variable livedo
  • Cutis marmorata telangiectatica congenita: congenital form of persistent livedo
  • Primary livedo reticularis: benign form of livedo of unknown cause
  • Secondary livedo reticularis: association of livedo with an underlying systemic disease
  • Livedo racemosa: generalised and persistent form of livedo 

What causes livedo reticularis? 

Livedo reticularis results from disturbance of blood flow to the skin causing low blood flow and reduced oxygen tension to the skin. Cutaneous vasculature is comprised of a series of 1–3 cm cones. The apex of each cone is located deep within the dermis at the site of an ascending arteriole. At the margin of each cone, the density of the arterial bed is decreased and the superficial venous plexus is more prominent.  Any pathological or physiological process which impairs blood flow to the skin results in higher amounts of deoxygenated haemoglobin, leading to a livid discoloration.

Physiological arteriolar vasospasm in response to cold produces reversible skin discolouration, such as in cutis marmorata. Prolonged vasospasm, thrombosis or hyperviscosity can cause the pathological skin changes of livedo racemosa. 

Who gets livedo reticularis?

Cutis marmorata  causes temporary or physiological livedo in about 50% of normal infants and many adults, particularly young women when exposed to the cold. 

Cutis marmorata telangiectatica congenita is a rare condition in which pronounced livedo is present at birth or soon after. It often improves with age. There are several congenital abnormalities associated with cutis marmorata telangiectatica.

 Primary livedo reticularis is the idiopathic form in adults, and can be persistent. The diagnosis is usually made once other more serious causes of livedo reticularis have been ruled out. 

Secondary livedo, or livedo racemosa, is associated with a range of systemic diseases. 

Obstruction / vasculopathy 

Occlusion of vessels may occur because of a hypercoagulable state.

  • Antiphospholipid syndrome (APS, also known as lupus anticoagulant syndrome) is characterised by blood clots due to sticky platelets. It results in livedo racemosa in 25% of patients, and in 70% of those with SLE-associated APS. 
  • Sneddon syndrome is an association of livedo racemosa with stroke in young adult women.
  • Livedoid vasculopathy is associated with ulceration due to fibrinolytic abnormalities and microcirculatory thrombosis.
  • Cryoglobulinaemia (immune globulins that precipitate in the cold)
  • Polycythaemia rubra vera (excessive number of red cells) or thrombocythaemia (platelet clumps)
  • Multiple myeloma
  • Cold agglutinin disease
  • Protein C and S deficiency
  • Antithrombin III deficiency
  • Disseminated intravascular coagulation
  • Haemolytic uraemic syndrome
  • Deep venous thrombosis

Autoimmune / vasculitis / connective tissue disease

Small, medium and large vessel vasculitis are associated with livedo racemosa.

Neurological disease

  • Reflex sympathetic dystrophy
  • Multiple sclerosis
  • Parkinson disease

Infection

Malignancy

Drugs

Amantadine (dopamine agonist used to treat Parkinson disease, multiple sclerosis and attention deficit hyperactivity disorder) causes livedo through arteriolar vasospasm associated with depletion of catecholamines.  Other drug-associated livedo has been reported with:

  • Minocycline  
  • Gemcitabine 
  • Catecholamines
  • Non-steroidal anti-inflammatories.

Other

What are the clinical features of livedo reticularis?

Livedo reticularis is characterised by:

  • Net or lace-like cyanotic pattern
  • Blotchy, reddish-blue to purple (livid colour)
  • Transient or persistent
  • Mostly affects legs
  • May be more pronounced in cold conditions
  • May or may not improve with warming 
  • Cold exposure may result in tingling and numbness of affected skin

In cutis marmorata, mottling is diffuse, mild and usually symptomless. The livedo commonly occurs on the legs and gradually resolves on rewarming. Cutis marmorata telangiectatica congenita is more pronounced than cutis marmorata. Primary livedo reticularis in adults is  intermittent, and the changes in skin colour are less influenced by ambient temperature.  

Cutis marmorata

Livedo racemosa often affects the trunk and buttocks as well as legs. The net-like violaceous pattern tends to be composed of irregular, broken macules with an annular pattern.. 

Livedo racemosa

 What are the complications of livedo reticularis?

Livedo reticularis itself is relatively benign. However, thromboembolic disease due to associated conditions such as antiphospholipid syndrome may lead to serious arterial events including death of the patient.

How is livedo reticularis diagnosed?

Livedo reticularis/racemosa is diagnosed by its clinical appearance. Investigations are undertaken to seek for an underlying cause (see above) based on careful history and examination, as extensive screening studies are unlikely to be helpful. The lupus anticoagulant panel should be ordered in acquired livedo that is not clearly induced by the cold.

If required, it is best to take at least 2 biopsies from red/blue and white areas of the livedo, and to ask for serial sections to be performed. Even a large skin biopsy of the centre of livedo racemosa ring may be reported as normal.  Multiple biopsies may increase the diagnostic yield. The goal of biopsy is to obtain samples of the medium vessel found in the deep reticular dermis and subcutanous fat, which may require a wedge or large punch biopsy for increased yield. 

Early signs of vasculopathy are lymphocytes and histiocytes attaching to endothelial cells (lining cells of the blood vessels). The cells join with fibrin to occlude the lumen of capillaries. Other capillaries may be dilated, or replaced by scar tissue (fibrosis).

What treatments are available for livedo reticularis?

There is no specific treatment for livedo reticularis, except for cold avoidance. 

In some patients the symptoms may improve spontaneously with age. Rewarming the area in idiopathic cases or treatment of the underlying cause of secondary livedo may reverse the discolouration. Treatment should be directed at treating the underlying disorder causing the livedo reticularis, if known.

Smoking cessation is essential to reduce risk of associated arterial events such as stroke.

What is the outlook for livedo reticularis?

Livedo reticularis itself is relatively benign. However, thromboembolic disease due to associated conditions such as antiphospholipid syndrome may lead to serious arterial events including death of the patient.

Cutis marmorata is usually less obvious with age. Over time, in primary livedo reticularis and livedo racemosa, the vessels become permanently dilated and livedo reticularis becomes permanent regardless of the surrounding temperature.

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Related information

 

References:

On DermNet NZ:

Other websites:

  • Search Medscape.com for articles about disorders that may cause livedo reticularis or livedo racemosa

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