Livedo reticularis

Livedo reticularis

Ruwani P. Katugampola and Andrew Y. Finlay

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports


Livedo reticularis (LR) is the net-like mottled violaceous discoloration of the skin secondary to dilatation and stagnation of blood within dermal capillaries. The unaffected normal colored islands of skin are the areas where blood supply is sufficient; in the network areas the supply is insufficient. This commonly occurs on the legs, arms, and trunk, but can be diffuse, and is more pronounced following exposure to cold. LR can be physiological (cutis marmorata), can occur as a primary phenomenon (idiopathic LR) or secondary to a number of diseases that cause dermal vessel wall thickening and/or lumen occlusion such as systemic lupus erythematosus, polyarteritis nodosa, anti-phospholipid syndrome (Chapter 16), cryoglobulinemia, oxolosis, cholesterol emboli, underlying malignancy (as a paraneoplastic phenomenon), and medications. Idiopathic LR may be congenital (cutis marmorata telangiectatica congenita), or associated with painful ulcers (see livedoid vasculitis chapter 134), or with cerebrovascular involvement (Sneddon syndrome).

Management strategy

The aetiology of physiological and primary LR is unknown, and no definitive treatment is available. The management of primary LR depends on the presence of associated ulcers (see livedoid vasculitis chapter), anomalies (congenital form), and systemic involvement (Sneddon syndrome). In secondary LR, the underlying cause needs to be identified and treated.

Physiological LR, which occurs in healthy children and adults in response to cold weather, is diffuse, mild, temporary, and usually asymptomatic. No specific treatment is required for this condition except avoidance of cold exposure, protection from cold exposure with warm clothing, and re-warming the affected area.

Cutis marmorata telangiectatica congenita is rare and presents at birth or soon after birth. A small proportion of affected children have associated congenital anomalies such as hemangiomas, glaucoma, limb atrophy, cardiac malformation or psychomotor retardation that need to be identified and referred for appropriate specialist care. The LR in these children usually spontaneously disappears or markedly improves with age.

Patients with Sneddon syndrome are at risk of cerebrovascular disease and may benefit from antithrombotic treatment. The timing of such treatment is debated as LR may precede the neurological events by up to 10 or more years. Advice regarding other risk factors predisposing to cerebrovascular events such as smoking, obesity, hypertension, and oral contraceptives is important.

Although a number of agents including anti-platelet therapy, danazole, pentoxifylline, and systemic steroids have been used to treat ulcers associated with LR, no single agent has been shown to completely resolve the LR itself.

Several medications, including those used in dermatology, have been associated with LR. The decision to withdraw the suspected medication should be based on the clinical judgment, alternative treatments and other side-effects rather than the appearance of LR per se. LR has been reported as a paraneoplastic phenomenon associated with metastatic breast cancer, renal cell carcinoma, and multiple myeloma.

Specific investigations

A detailed clinical history followed by physical examination is essential, especially in diagnosing Sneddon’s syndrome, identifying congenital anomalies in infants, and in excluding secondary causes of LR. The histology of LR is non-inflammatory thickening of dermal vessel walls with eventual occlusion of the lumen.

Aug 7, 2016 | Posted by in Dermatology | Comments Off on Livedo reticularis
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