Livedo reticularis (LR) is a vascular reaction pattern characterized by a net-like discoloration on the skin. LR may be physiologic, indicative of capillary vascular malformation, or associated with vasculitis or obstruction of flow. LR can be triggered by cold temperatures.

Cutis marmorata (CM) is a transient, benign (physiologic) presentation of LR that may also be seen in pale, gravid females.

Congenital LR, known as cutis marmorata telangiectatica congenita (CMTC), presents with persistent LR that can involve the trunk or extremities. Generally, the mottled discoloration is limited to one extremity but can be more generalized. There may be a sharp demarcation at midline with truncal involvement. CMTC is sporadically inherited (or autosomal dominant) and can be associated with limb, facial, and spine anomalies along with cutaneous atrophy. The vascular changes of the skin may improve during the first few years of life and resolve completely in approximately 20% of children with CMTC.

Many inherited vascular malformation syndromes are associated with CM or LR. LR is also associated with a host of other factors including anticardiolipin antibodies. It is seen with collagen vascular diseases such as Raynaud disease, systemic lupus erythematosus, dermatomyositis, scleroderma, polyarteritis nodosa, and temporal arteritis. In severe cases, the extremities are cold and ulcers may form.

Sneddon syndrome is extensive LR with central nervous system disease.

LR is also seen in patients with poor vascular flow due to peripheral vascular disease and cardiac failure, disseminated intravascular coagulation (DIC), deep vein thrombosis (DVT), vascular emboli, and vascular calcifications (in hypercalcemia). Infectious associations include syphilis, tuberculosis, streptococcemia, endocarditis, and rickettsial and viral diseases. Medication associations include amantadine, catecholamines, and quinidine; endocrine associations include hypothyroidism, pseudohypoparathyroidism, hypoparathyroidism, and Cushing syndrome.