Livedo reticularis in Child
Alerts and Notices
SynopsisLivedo 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.
R23.1 – Pallor
238772004 – Livedo reticularis
Differential Diagnosis & Pitfalls
- Telangiectases (unilateral nevoid telangiectasia) – Seen in adolescent girls and during pregnancy.
- Hereditary benign telangiectasia – S familial disorder; is associated with cutaneous and labial telangiectases.
- Adams-Oliver syndrome – Has CMTC and aplasia cutis congenital of the scalp, sometimes with bony defects.
- Ataxia-telangiectasia syndrome – Manifests during childhood with telangiectasias and cerebellar ataxia.
- Divry-Van Bogaert syndrome – A rare disorder with LR and leptomeningeal angiomatosis during adolescence.
- Osler-Weber-Rendu disease (HHT) – Includes significant visceral and cutaneous arteriovenous malformations and is inherited in an autosomal dominant pattern.
- When there is purpura in addition to underlying livedo, vascular disease or vasculitis (polyarteritis nodosa, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis) should be considered.
- Active ulceration or atrophic scars suggest underlying pathological disease.
- Amantadine and other drugs are associated with a livedo reticularis pattern.
- Angioma serpiginosum
- Emboli or thrombosis
- Serpentine supravenous hyperpigmentation (see drug-induced pigmentation)
Drug Reaction DataBelow is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
Livedo reticularis in Child