Livedo reticularis in Child
Alerts and Notices
Synopsis
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.
Codes
ICD10CM:R23.1 – Pallor
SNOMEDCT:
238772004 – Livedo reticularis
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Diagnostic Pearls
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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
- Hypercalcemia
- Oxalosis
- Serpentine supravenous hyperpigmentation (see drug-induced pigmentation)
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below 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.Subscription Required
References
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Last Reviewed:07/25/2018
Last Updated:01/18/2023
Last Updated:01/18/2023
Patient Information for Livedo reticularis in Child
Overview
Livedo reticularis is a harmless, lacy, symmetrical skin pattern of small blood vessels. It usually appears on the legs, especially when exposed to cold temperatures. It is caused by blood pooling in the veins. It can be associated with Raynaud syndrome, antiphospholipid syndrome, Sneddon syndrome, or lupus.Who’s At Risk
Young women (aged 20 to 40) exposed to cold temperatures, smoking, and stress may develop the distinctive lacy skin pattern of livedo reticularis.Some medications increase the risk of developing livedo reticularis.
Signs & Symptoms
Livedo reticularis is a reddish-blue symmetrical skin pattern most commonly seen on the legs. It may appear on the arms, chest, or abdomen. It gets worse when exposed to cold temperatures, and the skin feels cold to the touch.Self-Care Guidelines
Avoid tobacco use and wear warm clothing to protect skin from cold temperatures. If skin is cold, gradually rewarm exposed skin.Elevating the affected leg may help impaired circulation.
When to Seek Medical Care
Check with your doctor if you develop skin ulcers or symptoms of small blood vessel inflammation, such as:- Fever
- Headache
- Fatigue
- Weight loss
- General aches and pains
- Night sweats
- Numbness or weakness
Treatments
There is no general treatment for livedo reticularis, but your health care provider will want to find out if it is being caused by an underlying disease.If your livedo reticularis is caused by medication, your doctor may be able to reduce the medication or prescribe a substitute medication.
Livedo reticularis in Child