What is Livedo Reticularis?
Livedo reticularis refers to the mottled discoloration of the skin which can be seen in various conditions. Livedo reticularis resembles a lace or net in appearance and is a cyanotic or bluish discoloration which surrounds a pale central skin area.
The following terms are also included under the umbrella term of livedo reticularis:
Primary Livedo Reticularis: This is a benign type of skin discoloration with no known cause.
Cutis Marmorata Telangiectatica Congenita: This is a congenital type of skin discoloration.
Cutis Marmorata: This form of livedo reticularis is physiological in nature.
Secondary Livedo Reticularis: This type of skin discoloration occurs in relation with an underlying systemic disease
Livedo Racemosa: This type of skin discoloration is more generalized and stubborn type of livedo.
What are the Causes of Livedo Reticularis?
The cause of Livedo reticularis is any disturbance or hindrance of blood flow to the skin where there is reduced oxygen tension and decreased blood flow to the skin. This means that the skin does not receive oxygenated blood which causes a livid discoloration.
Vasospasm in the arteries occurring from cold produces skin discoloration or livedo, which is reversible.
Types of Livedo Reticularis
Cutis Marmorata: In this type of livedo reticularis, the patient has physiological discoloration of the skin, which is temporary, and can be seen in about half of the infants and also adults upon exposure to cold.
Cutis Marmorata Telangiectatica Congenital: This is a rare type of livedo where it is very much pronounced at birth or afterwards. This condition tends to improve with age. Patient also has various congenital abnormalities linked with cutis marmorata telangiectatica.
Primary Livedo Reticularis: This form of livedo reticularis is idiopathic in nature and can be persistent and is seen in adults. After serious causes are excluded, the diagnosis of primary livedo reticularis will be made.
Livedo Racemosa or Secondary Livedo: This type of livedo reticularis is associated with a variety of systemic diseases which include: vasculopathy, antiphospholipid syndrome, Sneddon syndrome, livedoid vasculopathy, cryoglobulinemia, multiple myeloma, Polycythaemia Rubra vera, cold agglutinin disease, hemolytic uremic syndrome, antithrombin III deficiency, polyarteritis nodosa, protein c and s deficiency, disseminated intravascular coagulation, deep venous thrombosis small, medium and large vessel vasculitis, dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, granulomatosis with polyangiitis and sjogren syndrome.
Infections such as syphilis, tuberculosis, mycoplasma pneumonia, bacterial sepsis, viral infection and rheumatic fever are also associated with secondary livedo.
Secondary Livedo is also seen in malignancies, such as inflammatory breast cancer, renal cell carcinoma, acute lymphocytic leukemia and lymphoma.
Medications which can also cause secondary livedo are: amantadine, gemcitabine, minocycline, non-steroidal anti-inflammatories and catecholamines.
Other causes of Secondary Livedo are: Septic emboli, cholesterol emboli , thromboangiitis obliterans, homocystinuria, hypercalcaemia, intra-arterial injection, primary fibromyalgia, pancreatitis and congenital hypogammaglobulinemia.
What are the Symptoms of Livedo Reticularis?
Common symptoms of Livedo Reticularis are:
- Reddish-blue to purple discoloration of skin, which is also blotchy in appearance.
- The discoloration of Livedo Reticularis has a lace or net-like pattern.
- Livedo Reticularis can be persistent or transient.
- Legs are commonly affected by Livedo Reticularis.
- Livedo Reticularis can be more pronounced in cold weather.
- Patient can also have numbness and tingling of the affected skin when it has occurred as a result of exposure to cold.
- Livedo Reticularis may or may not improve with warming.
- In cutis marmorata, the patient has more diffuse and mild mottling without any other symptoms. It is seen often on the legs and slowly subsides with re-warming.
- Cutis marmorata telangiectatica congenita is more distinct when compared to cutis marmorata.
- Livedo Racemosa commonly affects the trunk, legs and buttocks and the lace like discoloration consists of broken, irregular macules which has an annular pattern.
- Primary livedo reticularis is intermittent in adults and the color changes of the skin are not that influenced by temperature.
How is Diagnosis of Livedo Reticularis Made?
The diagnosis of Livedo reticularis or Livedo racemosa can be made just by looking at it. Different tests are done to look for the underlying cause of Livedo reticularis. The doctor can also order a lupus anticoagulant panel in patients with acquired livedo which has developed as a response to the cold.
Biopsies from the bluish-reddish and white areas of the livedo can be taken if needed. Serial sections need to be performed of the biopsy and multiple biopsies to help in accurate diagnosis of Livedo Reticularis.
What is the Treatment for Livedo Reticularis?
Livedo reticularis does not have any specific treatment, except for avoiding cold environments. Treating the underlying cause of secondary livedo reticularis will help in reversing the discoloration. Livedo reticularis can spontaneously improve in some patients with age. In some cases, re-warming the area of Livedo reticularis, where the cause is not known, helps. It is also important to stop smoking to cut down on the risk of related arterial events like stroke.
What is the Prognosis for Livedo Reticularis?
Livedo reticularis on its own is quite a benign condition. However, thromboembolic diseases from related conditions, such as antiphospholipid syndrome, can cause serious arterial events and can prove fatal for the patient. As a person ages, cutis marmorata becomes less obvious. In patients with livedo racemosa and primary livedo reticularis, gradually the vessels become permanently dilated and the livedo reticularis remains persistent regardless of the external temperature.