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Livedo Reticularis

Asphyxia Reticularis

Livedo reticularis is a type of skin condition that is characterized by purplish discoloration of skin, which occurs in patches. The parts of the body which are commonly affected are hands, arms, legs and feet.


Presentation

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].

Short Stature
  • stature) Primary hyperoxaluria, oxalosis (oxalate vasculopathy) Cytomegalovirus infection (very rare clinical form, presenting with persistent fever and livedo reticularis on the extremities and cutaneous necrotizing vasculitis of the toes) Generalized[en.wikipedia.org]
  • Nonetheless, FILS patients had congenital abnormalities, including mild facial dysmorphic features, immunodeficiency, livedo, and short stature.[doi.org]
Plethora
  • The use of dapsone may be associated with a plethora of adverse effects including rash but livedo reticularis has been very rarely reported.[ncbi.nlm.nih.gov]
Leg Edema
  • The diagnosis of AE in our patient with leg edema of unknown cause was considered prior to the appearance of any pruritic eruptions.[ncbi.nlm.nih.gov]
Movement Disorder
  • Magalhães, Tremor as the first neurological manifestation of Sneddon's syndrome, Movement Disorders, 20, 2, (248-251), (2004).[doi.org]
Abnormal Pulse
  • OBJECTIVE: To evaluate the prevalence of abnormal pulse wave velocity (PWV), pulse contour analysis (PCA) and abnormal ankle-brachial pressure index (ABPI) in patients with livedo reticularis (livedo) and without livedo.[ncbi.nlm.nih.gov]
  • D'Cruz, The prevalence of abnormal pulse wave velocity, pulse contour analysis and ankle-brachial index in patients with livedo reticularis: a controlled study, Rheumatology, 52, 11, (1992), (2013). L. Ruiz Gutiérrez and A.[doi.org]
Angioedema
  • The cause of angioedema with eosinophilia (AE) is unknown. Patients with AE sometimes develop pruritic eruptions or urticaria before the onset of edema.[ncbi.nlm.nih.gov]
Chronic Urticaria
  • Here, we report the case of a 45-year-old female prescribed dapsone for chronic urticaria after which she developed extensive livedo reticularis in the limbs, abdomen, and trunk.[ncbi.nlm.nih.gov]
Uremia
  • Patients typically have recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uremia. Oxalate can deposit in extrarenal sites such as the heart, walls of arteries and veins, bone, and skin.[ncbi.nlm.nih.gov]
Akinesia
  • In the last several years, amantadine has been increasingly prescribed for akinesia in Parkinson's disease and to combat fatigue associated with multiple sclerosis.[ncbi.nlm.nih.gov]
Tremor
  • Magalhães, Tremor as the first neurological manifestation of Sneddon's syndrome, Movement Disorders, 20, 2, (248-251), (2004).[doi.org]

Workup

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.

Nephrolithiasis
  • Patients typically have recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uremia. Oxalate can deposit in extrarenal sites such as the heart, walls of arteries and veins, bone, and skin.[ncbi.nlm.nih.gov]
Liver Biopsy
  • Renal biopsy, performed 3 days after presentation, suggested crystal deposition disease, and subsequent investigations, using both dialysate oxalate concentrations and liver biopsy, led to the diagnosis of primary hyperoxaluria (PH).[ncbi.nlm.nih.gov]

Treatment

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

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.

Etiology

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].

Epidemiology

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].

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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].

Summary

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].

Patient Information

  • 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. 

References

Article

  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.

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Last updated: 2019-07-11 21:21