Livedo reticularis I73.83

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 10.05.2024

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Synonym(s)

Cutis marorata; livedo anularis

Definition
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Harmless, functional, temperature-dependent (hypothermia) disturbance of the dermal blood supply with flow slowing and hypoxygenation of the blood of the affected region and livider reticulation of the skin. In contrast to the non-functional, but organically caused livedo racemosa, the reticulation consists of closed rings, which have a diameter of 2.0-3.0 cm. The red marginal zones do not correspond to vessels running through here, but mark the zones with low oxygenated blood. The feeding vessel of this skin compartment is located in the center of the ring (see figure with schematic drawing).

Remark: In livedo racemosa, on the other hand, vascular occlusion occurs with irregular focal circulatory disturbance, which leads to bizarre "lightning figure-like" erythema patterns on the skin (see figures).

Etiopathogenesis
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Mostly functional, harmless dysregulation usually occurring after exposure to cold (livedo reticularis e frigore; cutis marmorata) and disappearing after warming.

Less frequently after exposure to heat ( erythema e calore).

Furthermore, in:

  • blood viscosity disorders (polyglobulia, cryoglobulinemia; paraporteinemia).
  • central innervation disorders (apoplexy, trauma)
  • in case of infections (e.g. hepatitis C, tuberculosis)
  • in case of drug exposure (e.g. heparin, erythromycin, amantadine, interferons)
  • after carbon dioxide arteriography
  • in case of versch. Neoplasia (e.g. renal cell carcinoma).

Manifestation
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Regularly occurring in infants in the first weeks of life with slight cooling of the skin.

In adolescents and young (mostly female) adults as a harmless, functional disorder of blood circulation.

Livedo reticularis that occurs beyond the neonatal period can be a sign of trisomy 18, Down syndrome, the rare Cornelia de Lange syndrome or hypothyroidism.

Congenital livedo reticularis may be a cutaneous sign of livedo reticualris congenita megalencephaly syndrome, which is associated with Other manifestations may include facial dysmorphism, developmental delay, seizures and low muscle tone associated with mutations in the PIK3CA gene (PIK3CA stands for: Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha).

Localization
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Symmetrical at the extremities, possibly also at the trunk.

Clinical features
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Large-mesh livid marbling, depending on the shape disappearing or appearing after heating or cooling. Frequently peripheral flat acrocyanosis which dissolves proximally into the livid ring structures of the livedo reticularis.

Histology
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Only gradual changes to normal findings. Gfls. dilatation of the postcapillary venules of the superficial and deep vascular plexus. No vascular occlusion (DD Livedo racemosa).

Differential diagnosis
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Livedo racemosa (see also fig.): irregular bizarre redness, no closed erythema circles.

Acrocyanosis: areal, mostly blue--red discolorations. Skin cool.

Therapy
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Roborative measures such as alternating warm baths, sauna, dry brushing, etc..

Progression/forecast
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Improvement with age.

Literature
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  1. Gibbs MB et al (2005) Livedo reticularis: an update. J Am Acad Dermatol 52: 1009-1019
  2. Endo Y et al (2011) Idiopathic livedo reticularis concomitant with polyclonal IgM hypergammopathy. Eur J Dermatol 21:266-267
  3. Fox M et al (2012) Livedo reticularis: a side effect of interferon therapy in a pediatric patient with melanoma. Pediatric Dermatol 29:333-335
  4. Ruiz-Genao DP (2005) Livedo reticularis associated with interferon alpha therapy in two melanoma patients. J Eur Acad Dermatol Venereol 19: 252-254
  5. Sladden MJ et al (2003) Livedo reticularis induced by amantadine. Br J Dermatol 149: 656-658
  6. Zelger B et al (1995) Differential diagnosis of livedo syndromes. dermatologist 46: 369-379

Disclaimer

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

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Last updated on: 10.05.2024