Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis

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14 Citations (Scopus)

Abstract

A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.

Original languageEnglish
Pages (from-to)298-300
Number of pages3
JournalActa Dermato-Venereologica
Volume81
Issue number4
DOIs
Publication statusPublished - 2001

Fingerprint

Hereditary Angioedema Types I and II
Hereditary Angioedemas
Angioedema
Complement C1 Inhibitor Protein
Lymphoproliferative Disorders
Antimalarials
Autoimmune Diseases
Signs and Symptoms
Infection
Neoplasms
Erythema elevatum diutinum

Keywords

  • Acquired angiooedema
  • C1-esterase inhibitor deficiency
  • Vasculitis

ASJC Scopus subject areas

  • Dermatology

Cite this

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abstract = "A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.",
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T1 - Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis

AU - Farkas, H.

AU - Szongoth, M.

AU - Bély, M.

AU - Varga, L.

AU - Fekete, B.

AU - Karádi, I.

AU - Füst, G.

PY - 2001

Y1 - 2001

N2 - A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.

AB - A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.

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