Signs and symptoms of livedo reticualris significantly vary with the underlying causative factor. Development of rash mainly on the lower extremities is the major symptom of livedo reticularis. The rash develops in a net like pattern with mesh size less than 3 cm. The rash is purplish to reddish blue in color which no distinct border and does not itch. The joint of the affected leg or hand undergoes swelling and is painful. In many cases, this may be accompanied by tingling or numbness in the area. When left untreated, ulcers may develop at the site [7].

• Anemia

  Associated Disorders Livedo reticularis is known to occur in several different autoimmune diseases, including systemic lupus erythematosus (SLE), autoimmune hemolytic anemia, polyarteritis nodosa, dermatomyositis, rheumatoid arthritis cold agglutinin [elaine-moore.com]

  Discussion: Cold agglutinin autoimmune hemolytic anemia (AIHA) affects approximately 1 in 1 million individuals per year, females more frequently than males, and typically in the seventies. The mortality rate of autoimmune hemolytic anemia is 11%. [shmabstracts.com]

  A 60-year-old man developed truncal livedo reticularis and ischemic necrosis of the left foot associated with thrombocytopenia, disseminated intravascular coagulopathy (DIC), and microangiopathic hemolytic anemia during intravenous heparin therapy. [ncbi.nlm.nih.gov]

• Pallor

  Livedo reticularis is a spastic-anatomical condition of the small vessels which translates morphologically by a reticular pattern, interspersing cyanosis, pallor and erythema. The same can be congenital or acquired. [ncbi.nlm.nih.gov]

  2015 ICD-9-CM Diagnosis Code 782.61 Pallor 2015 Billable Thru Sept 30/2015 Non-Billable On/After Oct 1/2015 ICD-9-CM 782.61 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 782.61 should only be used [icd9data.com]

  Her fingers manifest the well-demarcated cold-associated pallor of Raynaud phenomenon (RP). The appearance is consistent with livedo reticularis (LR). [thoracickey.com]

  ICD-10-CM Codes › R00-R99 Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified › R20-R23 Symptoms and signs involving the skin and subcutaneous tissue › R23- Other skin changes › Pallor 2016 2017 2018 2019 Billable/Specific [icd10data.com]

• Splenectomy

  Case Presentation: A 65 year old female with a history of autoimmune hemolytic anemia (unknown type, but treated with rituximab in the past) and idiopathic thrombocytopenic purpura status post splenectomy was visiting from out of town when she presented [shmabstracts.com]

  Splenectomy, distal pancreatectomy and gastrectomy were performed. The biopsy revealed the presence of cholesterol crystal emboli (CCE) ( Fig. 2 ). Full patient recovery was achieved. The mortality rate of CCE can reach 65%. [medintensiva.org]

• Pneumonia

  […] to M. pneumonia [7], [8]. [ejdv.eg.net]

  Dermatomyositis Rheumatoid arthritis Polyarteritis nodosa Granulomatosis with polyangiitis Temporal arteritis Sjögren syndrome Neurological disease Reflex sympathetic dystrophy Multiple sclerosis Parkinson disease Infection Tuberculosis Syphilis Mycoplasma pneumonia [dermnetnz.org]

  Mandell, MD, PhD Cleveland Clinic Journal of Medicine November 2022, 89 (11) 607-608; DOI: https://doi.org/10.3949/ccjm.89b.11022 As newer vaccines against Streptococcus pneumoniae have arrived, so have confusing recommendations and guidelines for the [ccjm.org]

• Xerostomia

  […] suggestive of an associated collagen vascular disorder, which may include one of the following: - SLE: malar erythema, photosensitivity and photodistributed eruption, discoid lesions, alopecia, arthralgias - Sjögren's syndrome: keratoconjunctivitis, xerostomia [dermatologyadvisor.com]

  Keratoconjunctivitis, scaly annular erythema and xerostomia on face and neck are commonly associated with Sjögren’s syndrome Skin fibrosis and sclerosis, matted telangiectasias, Raynaud’s phenomenon, dilated capillary loops in nail fold and calcinosis [drugsdetails.com]

• Purpura

  The cutaneous features of cryoglobulinaemia, and the mechanism of the reticulate purpura in this condition, are discussed. [ncbi.nlm.nih.gov]

  If concurrent purpura, nodules, macules, ulcerations, and/or atrophie blanche are noted, a secondary cause of LR or livedo racemosa exists (Figure 75-4). [thoracickey.com]

  Localized livedo reticularis and retiform purpura are signs of occlusion of medium-size vessels of the skin. (1,2) Critical occlusion of medium-size vessels of the skin can result in retiform purpura and skin necrosis as well as necrosis of the fingers [kenkyugroup.org]

• Skin Ulcer

  Skin ulcers are commonly described in the juvenile form of dermatomyositis; however, in the adult form they are rarely described. [ncbi.nlm.nih.gov]

  Patients with livedo racemosa have a significantly higher frequency of skin ulcerations, arthralgias, cutaneous vasculitis, and higher c-reactive protein (crp) levels when compared to their lr counterparts.3 With exception of the characteristic skin changes [thoracickey.com]

  Other, less common skin conditions associated with APS include: skin ulcerations superficial thrombophlebitis gangrene Skin ulcerations Livedoid vasculopathy results in painful skin ulcers and scarring due to chronic clotting in the blood vessels of the [hss.edu]

  However, if blood flow is completely blocked, pain and skin ulcers can develop. Your health care provider will ask about your symptoms. Blood tests or a skin biopsy may be done to help diagnose any underlying health problem. [nlm.nih.gov]

• Skin Disease

  You may also need Related Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e [clinicalgate.com]

  Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 134. Livedo reticularis - close-up - illustration This is a close-up view of livedo reticularis. [stlukes-stl.com]

  If you are uncomfortable with the appearance of your skin, talk to your provider about treatment, such as taking medicines that can help with the skin discoloration. For secondary LR, treatment depends on the underlying disease. [nlm.nih.gov]

  Search for: LOGIN | CREATE ACCOUNT Contributing Editors Help About Us Store Twitter Facebook LOGIN | CREATE ACCOUNT Home Clinical Reference Discover Beta Videos Board Review Quizzes Mobile Apps For Your Patients A Clinical Atlas of 101 Common Skin Diseases [derm101.com]

• Malar Rash

  Common Lupus Skin Problems Malar Rash About half of all lupus patients experience a characteristic rash called the malar or “butterfly” rash that may occur spontaneously or after exposure to the sun. [hopkinslupus.org]

  She had a long history of systemic lupus erythematosus (SLE) that had started 26 years ago with malar rash and arthritis. Fig. 1. (a) Livedo reticularis on both lower limbs. [medicaljournals.se]

• Dizziness

  […] emergency and may be caused by: accident or trauma blood loss infection poison burns As well as cold, pale, or mottled skin, the symptoms of shock include: breathing difficulties enlarged pupils rapid heart rate rapid breathing nausea vomiting weakness dizziness [medicalnewstoday.com]

  Nonspecific prodromal symptoms (headache, dizziness) often precede livedo reticularis by several years and this is followed by the development of focal neurological symptoms and then progressive cognitive impairment. [patient.info]

  The symptoms of shock include: mottled, cold, or pale skin breathing problems larger-than-normal pupils rapid pulse rapid breathing nausea and vomiting weakness dizziness fainting Shock is a medical emergency that requires immediate medical attention. [healthline.com]

  Additional symptoms may include headache, dizziness, abnormally high blood pressure (hypertension), and heart disease. [rarediseases.org]

• Vertigo

  Headache and vertigo may precede the onset of livedo racemosa and cerebrovascular manifestations by several years. In the course of the disease, memory disturbances, personality changes, and cognitive decline leading to dementia occur frequently. [orpha.net]

  Benign paroxysmal positional vertigo: Effective diagnosis and treatment Shayna R. Cole, AuD, CCC-A and Julie A. [ccjm.org]

  Generalized symptoms (e.g., headaches and migraines and/or dizziness or vertigo) may be present for several years before neurological symptoms and/or visible skin discoloration appears. High blood pressure (hypertension) is also common. [rarediseases.org]

• Kidney Failure

  Some of the other possible causes of the disorder are mentioned below: Thrombocytosis Polycythaemia rubra vera Syphilis Tuberculosis Lyme disease Acute kidney failure Use of intravenous drugs and birth control pills Pancreatitis Intake of amantadine Regular [undiagnosedwarrior.org]

Diagnosis of livedo reticularis would depend on obtaining information about secondary ulceration, subcutaneous nodules and retiform purpura. A past medical history of the patient should be carefully noted. This would provide insight of underlying diseases if any. Following this, a detailed drug profile should also be taken. Physical examination of the characteristics of the rash is carried out. If the patient is suffering from fever, then it may be an indication of infection as the cause of livedo reticularis.

Laboratory tests such as complete blood count, proteinuria, kidney function tests, coagulation studies, antinuclear antibodies, antiphospholipid antibodies, antineutrophil cytoplasmic antibodies along with hepatitis B and C serology would be done. Skin biopsy confirms the condition of livedo reticularis.

• Streptococcus Pneumoniae

  Mandell, MD, PhD Cleveland Clinic Journal of Medicine November 2022, 89 (11) 607-608; DOI: https://doi.org/10.3949/ccjm.89b.11022 As newer vaccines against Streptococcus pneumoniae have arrived, so have confusing recommendations and guidelines for the [ccjm.org]

Treatment depends on treating the underlying disease condition and effective management of symptoms. Exercise such as leg elevation and ankle pumping helps in establishing appropriate blood circulation thereby relieving the constriction in blood vessels. Application of warm therapy to the affected area can also help. Individuals are advised against application of ice or any coolant to the area.

Antiinflammatory medications are administered if rheumatoid arthritis is the cause. Skin lesions are treated with low dose aspirin. Anticoagulant therapy and antiplatelet therapy are also indicated [8]. Another mode of treatment that is also quite effective in treatment of livedo reticularis is PUVA therapy [9].

Prognosis of the condition majorly depends on the underlying etiology. The discoloration of the skin and blood circulation may improve upon treatment. However, if idiopathic livedo reticularis has set in then skin discoloration may be permanent in nature.

Livedo reticularis occurs due to spasms in the blood vessels. Or it may also occur due to abnormality that occurs in the circulation near the surface of the skin. In many instances, extreme cold weather conditions can trigger an attack of livedo reticualris. However, it can also indicate the development of some serious underlying disease conditions, which include vascular disease, rheumatologic disease and endocrine disorders. Livedo reticularis can also occur as a side effect of certain medications such as amantadine given for treatment of Parkinson disease [2]. In rare cases, it can also occur as a secondary complication to dialysis; a condition known as calciphylaxis [3].

Livedo reticularis is a common occurrence amongst the female population. Patients suffering from antiphospholipid syndrome are at an increased risk of developing the skin condition. The condition was first described in the year 1970 by Shealy et al. It was estimated that, amantadine-induced livedo reticualris was prevalent in 2 to 28% of patients [4].

Livedo reticularis can be divided into 4 different categories based on the appearance of livedo and the underlying etiology. These include physiologic, primary, idiopathic, and amantadine-induced livedo reticularis. The primary form majorly strikes young women and affects the lower extremities. It gets exaggerated due to cold weather and gradually resolves on re-warming. The condition is also otherwise known as cutis marmorata.

The basic physiologic changes that take place for skin discoloration and mottling are due to contractions of blood vessels. Such an event restricts the blood supply to the affected area causing purplish or reddish blue discoloration of skin. The skin looks mottled and has a net-like pattern, which primarily occurs due to stagnation of deoxygenated blood.

In primary form, the change in skin color is not related to temperatures. Idiopathic livedo reticularis does not resolve and is a persistent form of skin condition. This may be an important marker for early stage of Sneddon’s syndrome. The condition may also occur as a secondary phenomenon to antiphospholipid syndrome and livedo vasculitis [5] [6].

Amantadine-induced livedo reticularis, as the name suggest occurs due to side effect of the medication amantadine which is used for treatment and management of diseases such as Parkinson disease and multiple sclerosis.

Exposure to extreme cold temperatures should be avoided to prevent development of livedo reticularis. Individuals with underlying disease condition should make all possible efforts to effectively manage their condition in order to avoid onset of livedo reticularis as a secondary complication [10].

Livedo reticularis may occur in healthy individuals or may even strike those who are suffering from underlying disease conditions. Lower extremities are affected the most. The condition gets severe in cold weather conditions. Discoloration of the skin primarily occurs due to swelling of the venules due to contractions. In such a type of condition, the skin assumes a net-like appearance with distinct borders and becomes mottled [1].

• Definition: Livedo reticularis is a type of skin disease, characterized by development of purplish to bluish discoloration of skin in a net-like pattern. In some cases, the discoloration may resolve when warm therapy is employed.
• Cause: Underlying disease condition is the major cause of livedo reticularis, which primarily occurs due to constriction of the blood vessels, restricting the blood flow to the affected area. The condition can also occur in response to extreme cold weather. In rare cases, livedo reticularis can occur as a secondary complication to kidney dialysis.
• Symptoms: Development of rash in the affected area is the characteristic symptom of livedo reticularis. There is net-like appearance in area along with purplish discoloration of the skin. The rash is non itchy and the joints in the affected hand or leg undergo swelling and are painful.
• Diagnosis: Various laboratory studies to analyze complete blood profile and kidney function tests are carried out. This is followed by coagulation studies, proteinuria, antinuclear antibodies, urinary sediment, cryofibrinogen levels, and hepatitis B and C serology. Along with these tests, skin biopsy is also done which confirms the findings.
• Treatment: Treatment of livedo reticularis is geared towards management of underlying disease condition and relieving the associated symptoms. Medications to relive arthritic pain are administered if rheumatoid arthritis is the underlying etiology. Application of warm therapy can help resolve the lesion in some cases. In addition, PUVA therapy and administration of low dose aspirin or anticoagulants are also found to be beneficial. 

1. Feldaker M, Hines EA Jr, Kierland RR. Livedo reticularis with summer ulcerations. AMA Arch Derm. Jul 1955;72(1):31-42.
2. Schwab RS, Poskanzer DC, England AC Jr, Young RR. Amantadine in Parkinson's disease. Review of more than two years' experience. JAMA 1972; 222:792.
3. Ledbetter LS, Khoshnevis MR, Hsu S. Calciphylaxis. Cutis. Jul 2000;66(1):49-51.
4. Browse NL, Burnand KG. The cause of venous ulceration. Lancet. Jul 31 1982;2(8292):243-5.
5. Nalli C, Andreoli L, Casu C, Tincani A. Management of recurrent thrombosis in antiphospholipid syndrome.Curr Rheumatol Rep. Mar 2014;16(3):405.
6. Bard JW, Winkelmann RK. Livedo vasculitis. Segmental hyalinizing vasculitis of the dermis. Arch Dermatol 1967; 96:489.
7. Abbade LP, Lastória S. Venous ulcer: epidemiology, physiopathology, diagnosis and treatment. Int J Dermatol. Jun 2005;44(6):449-56
8. Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol 1982; 7:359.
9. Tuchinda C, Leenutaphong V, Sudtim S, Lim HW. Refractory livedoid vasculitis responding to PUVA: a report of four cases. Photodermatol Photoimmunol Photomed 2005; 21:154.
10. Singh N, Armstrong DG, Lipsky BA. Preventing foot ulcers in patients with diabetes. JAMA. Jan 12 2005;293(2):217-28.