Livedo racemosa (overview) M30.8

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.).

• 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
  

• In the idiopathic Livedo racemosa no underlying diseases are found.
• Further described is the (polyätiogical) syndromal livedo racemosa in connection with:
  • Polyarteritis nodosa, endangiitis obliterans, bacterial endocarditis, lupus erythematosus, dermatomyositis, syphilis, tuberculosis, chronic polyarthritis (rheumatoid arthritis), arterial embolism(cholesterol embolism), cutaneous B-cell lymphoma, Cryoglobulinemia, intravascular coagulopathy, hypertension, pancreatitis, arteriosclerosis, cryoglobulinemia, intraarterial injections, pseudohyperparathyroidism, phospholipid-antibody syndrome, protein C deficiency, hepatitis, viral diseases.
• Pathogenetically, partial or complete occlusion of small and medium-sized vessels of the deep vascular plexus of the skin is caused by proliferation of smooth muscle and endothelial cells. Rheological causes (stasis, microthrombi).

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

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.

Thrombocytosis can be found.

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.

• Subtly clarify underlying disease, therapy according to underlying disease. Strict smoking ban, discontinuation of oral contraceptives and antiplatelet drugs (e.g. ASA).
• In case of severe pain symptoms, a trial with IVIG (0.5 g/kg bw, every 4 weeks) is recommended. In 2021, the treatment response to IVIG (2 g/kg bw for 5 days) was retrospectively investigated in a relatively large collective (Kofler et al.). This showed a good response and improvement in terms of ulceration, pain, and limitations in daily life.
• In the idiopathic form without further signs of disease, no further therapy is required, but close follow-up is necessary, since the symptom "livedo" may precede a vascular systemic disease.

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