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Livedo reticularis
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 netlike, 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 generalized (livedo racemosa) and is more pronounced after exposure to cold. LR can be physiologic (cutis marmorata), can occur as a primary phenomenon (idiopathic LR), or can be secondary to a number of diseases that cause dermal vessel wall thickening and/or lumen occlusion. Secondary causes of LR include infections (hepatitis B and C, HIV, meningococcus, mycoplasma, tuberculosis), systemic lupus erythematosus, polyarteritis nodosa, antiphospholipid syndrome, cryoglobulinemia, oxalosis, cholesterol emboli, hypercalcemia (secondary to malignancy, hyperparathyroidism, renal failure), underlying malignancy (as a paraneoplastic phenomenon), endocrinological crisis (pheochromocytoma), and medications. Idiopathic LR may be congenital (cutis marmorata telangiectatica congenita) or associated with painful ulcers (livedoid vasculitis) or with cerebrovascular involvement (Sneddon syndrome). LR has been reported as a paraneoplastic phenomenon associated with metastatic breast cancer, renal cell carcinoma, and multiple myeloma. LR has also been reported as a complication of accidental intraarterial filler injections, such as hyaluronic acid fillers and use of silicone implants for soft tissue augmentation.
Management Strategy
The etiology of physiologic and primary LR is unknown, and no definitive treatment is available. The management of primary LR depends on the presence of associated ulcers, anomalies (congenital form), and systemic involvement (Sneddon syndrome). In secondary LR, the underlying cause needs to be identified and treated.
Physiologic 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 rewarming 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 antiplatelet or antithrombotic treatment . The timing of such treatment is debated, as LR may precede the neurologic events by up to 10 or more years. Advice regarding decreasing or eliminating risk factors predisposing to cerebrovascular events such as smoking, obesity, hypertension, and oral contraceptives is important.
Although multiple agents, including antiplatelet and anticoagulant therapy, danazol, 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 clinical judgment, alternative treatments, and other side effects rather than the appearance of LR per se.
Specific Investigations
A detailed clinical history followed by physical examination is essential, especially in diagnosing Sneddon 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.
Livedo reticularis: a review of the literature
Sajjan VV, Lunge S, Swamy MB, et al. Indian Dermatol Online J 2015; 6: 315–21.
This review of LR includes an extensive list of its systemic associations. The authors conclude that management of secondary LR should be directed toward the underlying cause and that all patients with either primary or secondary LR should be encouraged to avoid the use of tobacco and vasoconstricting medications.
The histopathological characteristics of livedo reticularis
In SI, Han JH, Kang HY, et al. J Cutan Pathol 2009; 36: 1275–8.
A survey of 16 patients with LR concluded that multiple skin biopsies from both the peripheral erythematous and central whitish areas in LR increase diagnostic yield.
Diagnostic impact and sensitivity of skin biopsies in Sneddon’s syndrome. A report of 15 cases
Wohlrab J, Fisher M, Wolter M, et al. Br J Dermatol 2001; 145: 285–8.
Deep 4-mm punch biopsies from the central white part of the LR demonstrated a sensitivity of 27% with one biopsy, 53% with two biopsies, and 80% with three biopsies of diagnosing Sneddon syndrome in clinically suspected cases. The authors concluded that the positive histology was important for commencing prophylaxis of cerebrovascular events in patients initially presenting with LR.
Livedo reticularis: an underutilized diagnostic clue in cholesterol embolization syndrome
Chaudhary K, Wall BM, Rasberry RD. Am J Med Sci 2001; 321: 348–51.
Six out of eight patients with unexplained acute renal failure due to suspected cholesterol emboli syndrome (CES) demonstrated cholesterol emboli in skin biopsies of LR. Deep skin biopsies of LR are proposed as a safe diagnostic procedure to confirm CES, thus avoiding increased morbidity associated with biopsy of visceral organs.
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