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Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review.

Miranda AR, Ue AP, Sabbag DV, Furlani Wde J, Souza PK, Rotta O - An Bras Dermatol (2013 Jul-Aug)

Bottom Line: The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy.The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms.A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

View Article: PubMed Central - PubMed

Affiliation: Federal University of São Paulo, EPM, São PauloSPBrazil.

ABSTRACT
In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

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Related in: MedlinePlus

Severe edema on the upper lip, with deformation of the anatomy of the face
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f01: Severe edema on the upper lip, with deformation of the anatomy of the face

Mentions: 2. Female patient, 35 years, single, biomedical researcher, with a 12 years history ofrecurrent episodes of severe edema on the upper lip and face. Initially the attacksoccurred twice a year however last year these became more serious, occurred fortnightlyand with a duration of 4 to 5 days, including once requiring hospitalization in theIntensive Therapy Unit (Figures 1 and 2). The patient related the crisis to emotionalstress and also reported a worsening of symptoms during menstruation. She also neverbecame pregnant. The patient had been taking oral contraceptives for 12 years, but neverassociated it with the crisis of angioedema. She had previously used a large number ofantihistamines, corticosteroids and adrenaline, without improvement. Her mother hadsimilar episodes of angioedema for 28 years, but in the last eight years she did nothave more attacks. Diagnostic hypotheses of common and hereditary angioedema were madeand tests were requested for the diagnosis, during the inter-crisis and crisis period(Chart 1).


Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review.

Miranda AR, Ue AP, Sabbag DV, Furlani Wde J, Souza PK, Rotta O - An Bras Dermatol (2013 Jul-Aug)

Severe edema on the upper lip, with deformation of the anatomy of the face
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3760933&req=5

f01: Severe edema on the upper lip, with deformation of the anatomy of the face
Mentions: 2. Female patient, 35 years, single, biomedical researcher, with a 12 years history ofrecurrent episodes of severe edema on the upper lip and face. Initially the attacksoccurred twice a year however last year these became more serious, occurred fortnightlyand with a duration of 4 to 5 days, including once requiring hospitalization in theIntensive Therapy Unit (Figures 1 and 2). The patient related the crisis to emotionalstress and also reported a worsening of symptoms during menstruation. She also neverbecame pregnant. The patient had been taking oral contraceptives for 12 years, but neverassociated it with the crisis of angioedema. She had previously used a large number ofantihistamines, corticosteroids and adrenaline, without improvement. Her mother hadsimilar episodes of angioedema for 28 years, but in the last eight years she did nothave more attacks. Diagnostic hypotheses of common and hereditary angioedema were madeand tests were requested for the diagnosis, during the inter-crisis and crisis period(Chart 1).

Bottom Line: The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy.The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms.A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

View Article: PubMed Central - PubMed

Affiliation: Federal University of São Paulo, EPM, São PauloSPBrazil.

ABSTRACT
In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

Show MeSH
Related in: MedlinePlus