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Urticarial Vasculitis. A Review of the Literature

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Systemic Vasculitides: Current Status and Perspectives

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Abstract

Urticarial vasculitis (UV) is a clinical entity characterized by the aspects of leukocytoclastic vasculitis (LCV). Since its clinical features often overlap with those of common urticaria, a lesional skin biopsy is often necessary for the diagnosis. UV may be idiopathic or caused by endogenous (tumors) or exogenous (infections) factors. UV is frequently associated to consumption of serum complement factors. In this case, UV is called hypocomplementemic urticarial vasculitis (HUV). Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a more severe form characterized by hypocomplementemia and systemic symptoms. Some patients may have UV with normal complement levels (normocomplementemic UV: NUV) and they usually have minimal or no systemic involvement. Response of UV to treatment is variable.

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Abbreviations

UV:

urticarial vasculitis

LCV:

leukocytoclastic vasculitis

HUVS:

hypocomplementemic urticarial vasculitis syndrome

SLE:

systemic lupus erythematosus

NUV/HUV:

normocomplementemic/hypocomplementemic urticarial vasculitis

CU:

chronic urticaria

COPD:

chronic obstructive pulmonary disease

ESR:

erythrocytes sedimentation rate

ANA:

antinuclear antibodies

CAPS:

cryopyrin-associated periodic syndromes

FCAS:

familial cold autoinflammatory syndrome

MWS:

Muckle-Wells syndrome

CINCA:

chronic infantile neurological cutaneous and articular syndromes

NLRP3:

NOD-like receptor protein 3

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy

    Giulia De Feo, Roberta Parente, Chiara Cardamone & Massimo Triggiani

Authors
  1. Giulia De Feo
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  2. Roberta Parente
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  3. Chiara Cardamone
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  4. Massimo Triggiani
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Corresponding author

Correspondence to Massimo Triggiani .

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Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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© 2016 Springer International Publishing Switzerland

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De Feo, G., Parente, R., Cardamone, C., Triggiani, M. (2016). Urticarial Vasculitis. A Review of the Literature. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_28

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