Livedo reticularis in Adult
LR is exacerbated by cold temperatures. It may be physiologic (cutis marmorata), a primary disease (as in idiopathic cases), or it may be secondary to underlying disorders such as antiphospholipid antibody syndrome, vasculopathies, or autoimmune connective tissue disorders. Amantadine, quinidine, and catecholamines have also been described as triggers. Drugs used in the past to treat syphilis (bismuth and arsphenamine) also caused LR. Furthermore, infections can induce LR via the formation of cryoglobulins, cold agglutinins, septic emboli, immune vasculitis, or septic vasculitis. In adults, LR is frequently seen in patients with Raynaud phenomenon, in those with a predisposition to chilblains and acrocyanosis, and in patients with poor vascular flow (eg, peripheral vascular disease and cardiac failure).
These secondary forms may also present with livedo racemosa, where the reticular pattern is asymmetric, discontinuous, and localized. Localized forms may be associated with vasculitis. In severe cases, the extremities are cold and ulcers may form.
Sneddon syndrome is extensive diffuse LR or livedo racemosa with cerebrovascular disease (from transient ischemic attacks to frank cerebrovascular accidents), hypertension, and antiphospholipid antibodies.
R23.1 – Pallor
238772004 – Livedo reticularis
Differential Diagnosis & Pitfalls
- Erythema ab igne is a form of fixed reticulate dyspigmentation in skin with chronic repeated heat exposure.
- Cutis marmorata telangiectatica congenita often only involves one extremity although it can be more generalized. It is usually present at birth and may be associated with limb asymmetry, vascular anomalies, or neurologic abnormalities.
- Reticular erythematous mucinosis is a form of cutaneous mucinosis affecting the upper chest with prominent redness.
- Erythema infectiosum
- Erythema marginatum
- Lymphocytic macular arteritis
- Serpentine supravenous hyperpigmentation (see drug-induced pigmentation)
- Collagen vascular diseases or vasculitis (polyarteritis nodosa, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis)
- Hematologic or hypercoagulable conditions (antiphospholipid antibody syndrome, cryoglobulinemia, polycythemia vera, protein C or S deficiency, heparin-induced thrombocytopenia, thrombotic thrombocytopenic pururpa / hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, etc)
- Livedoid vasculopathy
- Embolic phenomena (cholesterol, fat, septic emboli)
- Deposition diseases (calciphylaxis, oxalosis)
- Medications (eg, amantadine, warfarin, interferon, minocycline, gemcitabine, quinidine)
- Infections (eg, hepatitis C, Mycoplasma, endocarditis, meningococcemia, syphilis, Rickettsia, M. leprae [Lucio phenomenon])
- Neoplasms (renal cell carcinoma, pheochromocytoma, some hematologic malignancies)
- Neurologic disorders (multiple sclerosis, Parkinson disease, reflex sympathetic dystrophy, Sneddon syndrome)
- Endocrine / metabolic conditions (hypercalcemia, hypothyroidism, carcinoid syndrome)
- Miscellaneous (chronic pancreatitis, heart failure, Degos disease)
- Pernio is cold-induced distal blisters or ulcers associated with cold and high humidity.
- Atrial myxoma
Drug Reaction Data