Livedo racemosa (overview) M30.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Inflammatio cutis racemosa; livedo racemosa generalisata; Livedovasculitis; livedo vasculitis (engl.); racemic vasculitis; reticular asphyxia multiplex

History
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Ehrmann, 1907

Definition
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The clinical picture of Livedo racemosa (see the different nomenclature in the Anglo-American literature) is a monitoring sign characterized by localized or generalized, rarely unilateral, bizarre, reticular ring structures (see fig.), reddish-livid skin markings which are not ring-like but open-worked (see fig.), and reddish-blue skin markings which show a red-blue coloration of varying intensity depending on the ambient temperature.

Pathophysiologically, the symptom "livedo racemosa" is caused by a focal slowing of the blood flow, resulting in an irregular, regionally varying oxygenation of the capillary blood (see below livedo).

In contrast to the functional livedo reticularis, the bizarre erythema pattern is based on organic, mostly thrombotic occlusions of skin vessels, which lead to the circulation disturbance of the skin areas. It is therefore always a vascular systemic disease that can affect the skin monoorganically, but can also affect other areas of care polyorganically (e.g. calciphylaxis, cholesterol embolism, antiphospholipid syndrome, Sneddon syndrome, etc.).

Classification
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  • Idiopathic livedo racemosa (monotopic or localized form): this form of livedo racemosa can be restricted to the skin as a monoorganic form. However, it can also change into the systemic form after a varying length of time.
  • Generalized livedo racemosa (Sneddon syndrome ): may develop from the idiopathic livedo racemosa. S.u. livedo syndromes
  • S.a. Livedovasculopathy
  • S.a. Hypertensive leg ulcer

Etiopathogenesis
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Localization
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Especially legs, upper arms, buttocks, back, front parts of the torso.

Clinical features
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Bizarrely shaped, flashing figure-like, striped or net-shaped, bluish-red, in the pattern not changing, localized skin discoloration. Slight fading under glass spatula pressure. Rare ulcerations.

Laboratory
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Thrombocytosis can be found.

Histology
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The (deep excision biopsy!) trial excision should include perilesional, clinically normal appearing skin. Complete and serial histological workup of the skin is required. Endangiitic changes with intimal growth and fibrinoid necrosis are found in the small arteries of the dermis and adjacent subcutaneous fatty tissue. Sectional obliteration of the vascular lumina. Sparse perivascular inflammatory infiltrates.

Differential diagnosis
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Clinical differential diagnosis:

  • Livedo reticularis (in Anglo-American usage called Livedo racemosa!): This functional disorder is best described by the term "Cutis marmorata". It is a cold-induced, large-meshed livid marbling, depending on the form disappearing or appearing after heating or cooling. Often peripheral acrocyanosis. The jagged "lightning figure-like" marking of the livedo racemosa (exclusion phenomenon) is always missing.
  • Livedovasculopathy: Reticular, deep red, livid, infiltrated erythema with formation of bizarre, usually very painful (not the livedo image but the stabbing permanent pain leads to the doctor! Ulcers are untypical for livedo racemosa (exclusion phenomenon).
  • Sneddon syndrome: livedo racemosa can be a monitoring sign of a potentially life-threatening, systemic, thrombotic vasculopathy (triad: livedo racemosa, zebrovascular insults with neuropsychiatric abnormalities, labile arterial hypertension).

  • Poly- (Peri-)arteriitis nodosa: 1.0 - 5.0 cm large, initially rough, usually very pressure dolent, also spontaneously painful, reddish to livid coloured plaques or nodules (iceberg phenomenon) with a tendency to painful ulceration. A live image like a flash can be part of the clinical symptoms.
  • Embolia cutis medicamentosa: Acute event, minutes to a few hours after an i.m. injection (anamnesis leads to a diagnosis of exclusion!), painful, hard infiltration with a figure-like skin pattern. Secondary: Development of deep ulcerations which heal with bizarrely shaped atrophic scars.
  • Arteriosclerotic occlusive ulcers (see below hypertonic leg ulcer): Not infrequently grafting on the clinical picture of livedo racemosa (here underlying arteriolosclerosis), development of haemorrhagic, jagged, very painful ulcers. Always evidence of peripheral AVK.
  • Cutis marmorata teleangiectatica congenita: Congenital clinical picture (exclusion criterion) with asymmetrically distributed Cutis marmorata with telangiectasia and phlebectasia; often conspicuously thin, translucent (atrophic) skin with clear vein pattern.
  • Calciphylaxis: Localized, mostly symmetrical picture of the razemose (lightning figure-like) livedo; this is accompanied by linear or also flat, 2.0 to 20 cm large, eminently painful, hard, red or also skin-colored plaques or nodules. Usually also painful ulcers of various sizes.

Histological differential diagnosis:

  • Livedo reticularis: Only gradual changes to normal findings. Gfls: Dilatation of the postcapillary venules of the superficial and deep vascular plexus.
  • Calciphylaxis: Thrombosed dermal and subcutaneous vessels with pronounced basophilic calcium deposits in the lumen and walls (exclusion phenomenon).
  • Dermatoliposclerosis (congestive dermatosis): Intralobular lipomembranous (membranocytic) fat necrosis and pronounced septal sclerosis. In advanced stages of dermatoliposclerosis, broad sclerosed septums with English wall-thickened capillaries and venules with PAS-positive perivascular fibrin sheaths, as well as macrophages and fibroblasts are found. In the dermis: Perivascular superficial and deep sclerosing dermatitis with wall thickened, glomeruli-like bulging vessels.
  • Leucocytoclastic vasculitis: signs of vascular wall necrosis with neutrophil infiltration and perivascular nuclear dust.
  • Thrombophlebitis migrans: Sclerosing endophlebitis (giant cell vasculitis) with complete or partial closure of the vascular lumina by thrombi. Frequent detection of giant cells.

Therapy
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  • Subtle clarification of the underlying disease, therapy according to the underlying disease. Strict smoking ban, discontinuation of oral contraceptives and thrombocyte aggregation inhibitors (e.g. ASS).
  • If pain symptoms are severe, IVIG (0.5 g/kg bw, every 4 weeks) is recommended.
  • In the idiopathic form without further signs of disease, no further therapy is required, but close monitoring of the course of the disease is necessary, as the symptom "livedo" can precede a vascular systemic disease.

Progression/forecast
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Favourable in the case of purely cutaneous infestation; otherwise unfavourable through concomitant infestation of the heart, kidney and brain (infarction, apoplexy), as progression cannot be stopped.

Literature
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  1. Baumann M et al (2000) Livedo racemosa: an unusual late manifestation of borreliosis? dermatologist 51: 593-596
  2. Champion RH (1965) Livedo reticularis. A review. Brit J Dermatol 77: 167-179
  3. Criado PR et al (2016) Epidemiological, clinicaland laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. Autoimmune Rev 15:558-563.
  4. Criado PR et al.(2016) Amantadine-Induced Livedo Racemosa.
    Int J Low Extreme Wounds 15:78-81.
  5. Degitz K et al (1989) Livedo racemosa in the detection of anti-phospholipid antibodies. Dermatologist 40: 437-440

  6. Ehrmann S (1907) A new vascular symptom in Lues. Vienna Med Weekly 57: 777-782
  7. Henschel A et al (1999) Disseminated ischemic necrosis and livedo racemosa in a chronic dialysis patient with calciphylaxis. dermatologist 50: 439-444
  8. Jorquera-Barquero E et al (2013) Oxalosis and livedo reticularis.Actas Dermosifiliogr 104:815-818
  9. March WC, Muckelmann R (1985) Generalized racemosis livedo with cerebrovascular lesions (Sneddon syndrome): an occlusive arteriopathy due to proliferation and migration of smooth muscle cells. 112: 703-708
  10. Mondal R et al.(2014) Childhood Polyarteritis Nodosa: a prospective multicentre study from easternIndia
    . Indian J Pediatr 81:371-374
  11. Relia N et al(2012) Monoclonal cryoglobulinemia, livedo reticularis, and renal failure. Kidney Int 82:118
  12. Schanz S et al (2003) Intravenous immunoglobulin in livedo vasculitis: a new treatment option? J Am Acad Dermatol 49: 555-556
  13. Weir NU et al (1995) Livedo reticularis associated with hereditary protein C deficiency and recurrent thromboembolism. Br J Dermatol 132: 283-285
  14. Wu S et al (2014) Sneddon's syndrome: a comprehensive review of the literature. Orphanet J Rare Dis 9:215.
  15. Zelger B et al (1995) Differential diagnosis of livedo syndromes. dermatologist 46: 369-379
  16. Zelger B et al (1992) Life history of cutaneous vascular lesions in Sneddon's syndrome. Hum Pathol 23: 668-675

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020