Introduction
Livedo reticularis is a cutaneous condition characterized by cyanotic skin discoloration called the net-like pattern in the reticular. This is a physical sign of disturbances in the cutaneous blood flow. Livedo reticularis occurs with a variety of physiological and pathological factors. Dark bluish discoloration of the skin in livedo reticularis is due to the reduced blood flow and low oxygen level at the skin peripheries. The characteristic features of livedo reticularis can be either transient or persistent.
What Is Livedo Reticularis?
Livedo reticularis is the reddish-blue skin discoloration in a net-like or lace-like pattern. The term livedo indicates the violet discoloration of the skin due to disturbances in the blood supply. Reticularis refers to a net-like or lace-like pattern of cyanotic discoloration. Livedo reticularis occurs in various conditions, including both physiologic and pathologic conditions. Young and middle-aged women are most prone to affect by livedo reticularis.
What Are the Types of Livedo Reticularis?
Based on the duration of the livedo, called the violet discoloration pattern, and the temperature, the livedo reticularis is divided into four types. The four types of livedo reticularis are physiologic livedo reticularis, primary livedo reticularis, Idiopathic livedo reticularis, Amantadine induced livedo reticularis.
Physiologic Livedo Reticularis: also known as cutis marmorata. This type of livedo reticularis occurs due to exposure to cold temperature; blood supply is disturbed by the excess cold temperature resulting in cyanotic discoloration of the skin. The symptoms will get resolved with an increase in temperature. Young women are mostly affected by physiologic livedo reticularis. The most commonly affected area is the leg.
Primary Livedo Reticularis: It is a benign form of livedo reticularis with discoloration of the skin with unknown cause. Idiopathic livedo reticularis is the early stage of Sneddon syndrome, antiphospholipid syndrome, or APS. Amantadine-induced livedo reticularis: livedo reticularis is caused by amantadine. The catecholamines provoke arteriolar vasospasm.
Other varieties of livedo reticularis include,
Cutis Marmorata Telangiectatica Congenita or CMTC: It is the congenital form of livedo reticularis. Features are persistent.
Livedo Racemosa: It is a persistent and generalized variety of livedo reticularis.
Secondary Livedo Reticularis: Livedo reticularis is caused due to underlying systemic diseases.
What Does Livedo Reticularis Look Like?
Livedo reticularis is characterized as the reddish-blue discoloration of the skin with a net-like or lace-like cyanotic pattern. Features can be either transient or persistent. In physiologic livedo reticularis, symptoms are seen due to the decreased temperature, which gets resolved in warming. The livedo reticularis affect the legs; sometimes, the trunk and buttocks will also get affected. Livedo racemosa has irregular macules with an annular pattern. Symptoms like tingling and numbness are present in cutis marmorata. Infants and young to middle-aged women are more prone to affect by cutis marmorata.
What Causes Livedo Reticularis?
The various physiologic and pathological processes that impair the blood supply of the skin cause cyanotic discoloration of the skin. An increased proportion of deoxygenated hemoglobin causes the livid coloration of the skin in livedo reticularis. In cutis marmorata, peripheral vasospasm occurs due to cold exposure. The cause of the idiopathic and primary type of livedo reticularis is unknown. vasospasm, thrombosis, and hyperviscosity cause pathologic skin changes in livedo racemose. In antiphospholipid syndrome, hypoxia and hypercoagulability cause livid skin changes. Certain medications such as Amantadine and other conditions like livedoid vasculopathy, primary fibromyalgia, and congenital hypogammaglobulinemia cause livedo reticularis.
What Are the Conditions Associated With Livedo Reticularis?
Cold exposure causes the cutis marmorata. Secondary livedo reticularis occurs due to the underlying conditions and disease. It is also stated that nutritional inefficiency can also cause livedo reticularis. Pellagra is the condition associated with secondary livedo reticularis.
Several other conditions associated with livedo reticularis are mentioned below.
Congenital Conditions :
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Cutis marmorata telangiectatica congenita or CMTC.
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Vasculitis.
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Transplacental transient.
Hematological and Hypercoagulability Condition:
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Antiphospholipid syndrome.
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Sneddon's syndrome.
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Cryoglobulinemia.
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Multiple myeloma.
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Cold agglutinin.
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Polycythemia vera.
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Deep venous thrombosis.
Autoimmune Disorders:
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Rheumatoid arthritis.
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Cutaneous polyarteritis nodosa.
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Wegener's granulomatosis.
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Takayasu arteritis. Large vessel vasculitis.
Neoplastic Conditions:
Lymphoma:
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Mycosis fungoides.
Connective Tissue Disorders:
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Dermatomyositis.
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Systemic lupus erythematosus.
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Sjogren's syndrome.
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Systemic sclerosis.
Infective Patients:
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Hepatitis C.
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Brucella.
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Endocarditis.
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Rheumatic fever.
Neurological Disorders:
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Diabetes mellitus.
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Pernicious anemia.
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Poliomyelitis.
Medications:
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Amantadine.
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Minocycline.
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Quinidine.
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Bismuth.
What Is the Differential Diagnosis of Livedo Reticularis?
Signs and symptoms of livedo reticularis are similar to other dermal disorders. Therefore, the comparison of livedo reticularis with similar conditions helps in proper diagnosis and aids in the differential diagnosis. The differential diagnosis of livedo reticularis is pointed out below.
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Erythema ab igne.
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Reticulated erythematous mucinosis.
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Poikiloderma.
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Mycosis fungoides.
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Dermatomyositis.
What Is the Histopathology of Livedo Reticularis?
In physiologic livedo reticularis, there is no change in the histopathology findings. Symptoms will get resolved with an increase in temperature. Other histopathological findings include calciphylaxis (calcium deposits inside the blood vessel), monoclonal cryoglobulinemia (eosinophilic plugging), cholesterol emboli, and oxalosis (crystal deposition) is present. Affected arterioles will show these changes. Histopathological findings are observed with the large elliptical biopsy taken from the center of the affected net pattern to the normal discolored area.
How to Diagnose Livedo Reticularis?
Livedo reticularis is diagnosed with a detailed history and physical examination. The history of the patients must include the location, exacerbation of symptoms and alleviating factors, symptoms duration, and skin changes. Personal and family history is also taken for any systemic diseases. A general systemic examination is also done. Medical history is also obtained for renal failure, and the patient's drug history is also considered. Laboratory findings should be correlated with personal and physical examinations. Skin biopsies are useful in diagnosing livedo reticularis. Multiple biopsies are made to improve the quality of diagnosis. Wedge biopsy or large punch cut biopsy is useful.
Diagnosis for livedo reticularis includes those mentioned below.
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A detailed history of the patient.
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Proper physical examination:
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Location.
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Symptoms.
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Alleviating factors.
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Duration of systems.
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Skin changes.
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Personal history.
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Family history.
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Skin biopsy:
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Multiple biopsies,
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Wedge biopsy.
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Large punch cut biopsy.
What Is the Treatment of Livedo Reticularis?
Livedo reticularis, due to exposure to cold, requires no treatment. Warming is required to resolve the symptoms in cutis marmorata. Vasodilator therapy is advised to improve the cosmesis. For secondary livedo reticularis with underlying systemic conditions, Treatment for the underlying disease is required. Medications include anticoagulants, antiplatelet agents, immunosuppressants, Pentoxifylline, Danazol, and tissue plasminogen activators can be advised. Hypoxic conditions can be advised with livedoid vasculopathy, hyperbaric oxygen and psoralen, and ultraviolet light A or PUVA therapy.
Conclusion
Livedo reticularis is a cutaneous condition that is challenging to diagnose. Primary livedo reticularis due to cold exposure usually needs no treatment. Avoiding exposure to cold conditions can be suggested to prevent physiological livedo reticularis. Behavior modification and limp elevation are helpful in symptomatic patients. However, in secondary livedo reticularis, the underlying disease is treated to resolve the symptoms of livedo reticularis.
