Livedo reticularis


Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by reduction in blood flow through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration. This can be a secondary effect of a condition that increases a person's risk of forming blood clots, including a wide array of pathological and nonpathological conditions. Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.
The condition may be normal or related to more severe underlying pathology. Its differential diagnosis is broadly divided into possible blood diseases, autoimmune, cardiovascular diseases, cancers, and endocrine disorders. It can usually be diagnosed by biopsy.
It may be aggravated by exposure to cold, and occurs most often in the lower extremities.
The condition's name derives.

Causes

A number of conditions may cause the appearance of livedo reticularis:
  • Cutis marmorata telangiectatica congenita, a rare congenital condition
  • Sneddon syndrome – association of livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause
  • Idiopathic livedo reticularis – the most common form of livedo reticularis, completely benign condition of unknown cause affecting mostly young women during the winter: It is a lacy purple appearance of skin in extremities due to sluggish venous blood flow. It may be mild, but ulceration may occur later in the summer.
  • Secondary livedo reticularis:
  • *Vasculitis autoimmune conditions:
  • **Livedoid vasculitis – with painful ulceration occurring in the lower legs
  • **Polyarteritis nodosa
  • **Systemic lupus erythematosus
  • **Dermatomyositis
  • **Rheumatoid arthritis
  • **Lymphoma
  • **Pancreatitis
  • **Chronic pancreatitis
  • **Tuberculosis
  • *Drug-related:
  • **Adderall
  • **Amantadine
  • **Bromocriptine
  • **Beta interferon treatment, e.g. in multiple sclerosis
  • **Livedo reticularis associated with rasagiline
  • **Methylphenidate and dextroamphetamine-induced peripheral vasculopathy
  • **Gefitinib
  • *Obstruction of capillaries:
  • **Cryoglobulinaemia – proteins in the blood that clump together in cold conditions
  • **Antiphospholipid syndrome due to small blood clots
  • **Hypercalcaemia
  • **Haematological disorders of polycythaemia rubra vera or thrombocytosis
  • **Infections
  • **Associated with acute kidney injury due to cholesterol emboli status after cardiac catheterization
  • **Arteriosclerosis and homocystinuria
  • **Intra-arterial injection
  • *Ehlers-Danlos syndrome – connective tissue disorder, often with many secondary conditions, may be present in all types
  • *Pheochromocytoma
  • *Livedoid vasculopathy and its association with factor V Leiden mutation
  • *FILS syndrome
  • *Primary hyperoxaluria, oxalosis
  • *Cytomegalovirus infection
  • *Generalized livedo reticularis induced by silicone implants for soft tissue augmentation
  • *As a rare skin finding in children with Down syndrome
  • *Idiopathic livedo reticularis with polyclonal IgM hypergammopathy
  • *CO2 angiography
  • *A less common skin lesion of eosinophilic granulomatosis with polyangiitis
  • *Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syndrome
  • *Livedo vasculopathy associated with IgM antiphosphatidylserine-prothrombin complex antibody
  • *Livedo vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity and prothrombin G20210A heterozygosity
  • *As a first sign of metastatic breast carcinoma
  • *Livedo reticularis associated with renal cell carcinoma
  • *Buerger's disease
  • *As a rare manifestation of Graves hyperthyroidism
  • *Associated with pernicious anaemia
  • *Moyamoya disease
  • *Associated with the use of a midline catheter due to angioplasty
  • *Familial primary cryofibrinogenemia.

    Diagnosis

Livedo reticularis is diagnosed by its clinical appearance and history. No further test or examination confirms idiopathic livedo reticularis. However, further investigations may be undertaken where an underlying cause is suspected such as skin biopsies, or blood tests for antibodies associated with antiphospholipid syndrome or systemic lupus erythematosus.

Treatment

Other than identifying and treating any underlying conditions in secondary livedo, idiopathic livedo reticularis may improve with warming the area.