Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema
Background: Hereditary angioedema (HAE) is a rare but life-threatening condition. HAE types I and II (HAE-1/2) result from C1-inhibitor (C1–INH) deficiency. However, recent genetic analysis has established a new type of HAE with normal C1–INH (HAEnC1-INH). The mutations of factor XII, plasminogen, a...
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Elsevier
2021-02-01
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Series: | World Allergy Organization Journal |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S1939455121000053 |
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Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Isao Ohsawa Atsushi Fukunaga Shinya Imamura Kazumasa Iwamoto Akio Tanaka Michihiro Hide Daisuke Honda Kouhei Yamashita Chisako Fujiwara Osamu Ishikawa Takeo Yamaguchi Junichi Maehara Tomoya Hirose Masahiro Ieko Kunihiko Umekita Yuya Nakamura Hiromichi Gotoh |
spellingShingle |
Isao Ohsawa Atsushi Fukunaga Shinya Imamura Kazumasa Iwamoto Akio Tanaka Michihiro Hide Daisuke Honda Kouhei Yamashita Chisako Fujiwara Osamu Ishikawa Takeo Yamaguchi Junichi Maehara Tomoya Hirose Masahiro Ieko Kunihiko Umekita Yuya Nakamura Hiromichi Gotoh Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema World Allergy Organization Journal Hereditary angioedema Prodromal symptom Erythema marginatum Bradykinin C1-inhibitor Icatibant |
author_facet |
Isao Ohsawa Atsushi Fukunaga Shinya Imamura Kazumasa Iwamoto Akio Tanaka Michihiro Hide Daisuke Honda Kouhei Yamashita Chisako Fujiwara Osamu Ishikawa Takeo Yamaguchi Junichi Maehara Tomoya Hirose Masahiro Ieko Kunihiko Umekita Yuya Nakamura Hiromichi Gotoh |
author_sort |
Isao Ohsawa |
title |
Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema |
title_short |
Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema |
title_full |
Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema |
title_fullStr |
Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema |
title_full_unstemmed |
Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema |
title_sort |
survey of actual conditions of erythema marginatum as a prodromal symptom in japanese patients with hereditary angioedema |
publisher |
Elsevier |
series |
World Allergy Organization Journal |
issn |
1939-4551 |
publishDate |
2021-02-01 |
description |
Background: Hereditary angioedema (HAE) is a rare but life-threatening condition. HAE types I and II (HAE-1/2) result from C1-inhibitor (C1–INH) deficiency. However, recent genetic analysis has established a new type of HAE with normal C1–INH (HAEnC1-INH). The mutations of factor XII, plasminogen, angiopoietin 1, and kininogen 1 genes may be the cause of HAEnC1-INH. Nevertheless, other causative molecules (HAE-unknown) may be involved. The Japanese therapeutic environment for HAE has been improving owing to the self-subcutaneous injection of icatibant, which was approved for the treatment of acute attack and enables early therapy. Erythema marginatum (EM) is a visible prodromal symptom which occasionally occurs prior to an angioedema attack; hence, recognizing the risk of an acute attack is important for early treatment. However, the detailed characteristics of EM remain unclear. In this study, we first investigated the clinical manifestations of EM in Japanese patients with HAE. Methods: A 20-point survey was developed and distributed to 40 physicians to gather clinical data on EM from patients with HAE. Results: Data on 68 patients with HAE (58 patients with HAE-1/2 and 10 patients with HAE-unknown) were collected. Of the patients with HAE-1/2, 53.4% experienced EM, whereas 43.1% did not. The forearm was the most frequent area of EM (64.5%), followed by the abdomen (29.0%) and upper arm and precordium (19.3%). Of the HAE-1/2 patients with EM, 41.9% always had angioedema following EM, while 29.0% always had colocalization of EM with angioedema. Moreover, 3.2% showed a correlation between the awareness of EM and severity of an angioedema attack. In 60.9% of HAE-1/2 patients with EM, the interval between the awareness of EM and appearance of angioedema was <3 h. Of the patients with HAE-unknown, 30.0% also experienced EM. Conclusion: We confirmed that more than one-half of Japanese patients with HAE-1/2 and one-third of those with HAE-unknown develop EM as the prodromal symptom of an angioedema attack. Physicians should communicate the significance of EM to patients with HAE to prepare them for possible imminent attacks. |
topic |
Hereditary angioedema Prodromal symptom Erythema marginatum Bradykinin C1-inhibitor Icatibant |
url |
http://www.sciencedirect.com/science/article/pii/S1939455121000053 |
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doaj-ee7b812df59041f487c84ec1e836eea02021-03-23T04:12:12ZengElsevierWorld Allergy Organization Journal1939-45512021-02-01142100511Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedemaIsao Ohsawa0Atsushi Fukunaga1Shinya Imamura2Kazumasa Iwamoto3Akio Tanaka4Michihiro Hide5Daisuke Honda6Kouhei Yamashita7Chisako Fujiwara8Osamu Ishikawa9Takeo Yamaguchi10Junichi Maehara11Tomoya Hirose12Masahiro Ieko13Kunihiko Umekita14Yuya Nakamura15Hiromichi Gotoh16Department of Nephrology, Internal Medicine, Saiyu Soka Hospital, Soka City, Saitama, Japan; Department of Nephrology, Juntendo University Faculty of Medicine, Bunkyo-ku, Tokyo, Japan; Corresponding author. Division of Nephrology, Internal Medicine, Saiyu Soka Hospital 1-7-22, Matsubara, Soka, Saitama, 340−0041, JapanDivision of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Hyogo, JapanDivision of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Hyogo, JapanDepartment of Dermatology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima City, Hiroshima, JapanDepartment of Dermatology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima City, Hiroshima, JapanDepartment of Dermatology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima City, Hiroshima, JapanDepartment of Nephrology, Juntendo University Faculty of Medicine, Bunkyo-ku, Tokyo, JapanDepartment of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Sakyo-ku, Kyoto, JapanDepartment of Dermatology, Gunma University Graduate School of Medicine, Maebashi City, Gunma, JapanDepartment of Dermatology, Gunma University Graduate School of Medicine, Maebashi City, Gunma, JapanDepartment of Gastroenterology, Japanese Red Cross Nagoya Daiichi Hospital, Nagoya City, Nagoya, JapanDepartment of Acute Care and General Medicine, Saiseikai Kumamoto Hospital, Kumamoto City, Kumamoto JapanDepartment of Traumatology and Acute Critical Medicine, Osaka University Graduate School of Medicine, Suita City, Osaka Japan; Emergency and Critical Care Medical Center, Osaka Police Hospital, Osaka City, Osaka, JapanDepartment of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, Ishikari-Tobetsu, Hokkaido, JapanDepartment of Rheumatology, Infectious Diseases and Laboratory Medicine, University of Miyazaki, Miyazaki City, Miyazaki, JapanDepartment of Nephrology, Internal Medicine, Saiyu Soka Hospital, Soka City, Saitama, JapanDepartment of Nephrology, Internal Medicine, Saiyu Soka Hospital, Soka City, Saitama, JapanBackground: Hereditary angioedema (HAE) is a rare but life-threatening condition. HAE types I and II (HAE-1/2) result from C1-inhibitor (C1–INH) deficiency. However, recent genetic analysis has established a new type of HAE with normal C1–INH (HAEnC1-INH). The mutations of factor XII, plasminogen, angiopoietin 1, and kininogen 1 genes may be the cause of HAEnC1-INH. Nevertheless, other causative molecules (HAE-unknown) may be involved. The Japanese therapeutic environment for HAE has been improving owing to the self-subcutaneous injection of icatibant, which was approved for the treatment of acute attack and enables early therapy. Erythema marginatum (EM) is a visible prodromal symptom which occasionally occurs prior to an angioedema attack; hence, recognizing the risk of an acute attack is important for early treatment. However, the detailed characteristics of EM remain unclear. In this study, we first investigated the clinical manifestations of EM in Japanese patients with HAE. Methods: A 20-point survey was developed and distributed to 40 physicians to gather clinical data on EM from patients with HAE. Results: Data on 68 patients with HAE (58 patients with HAE-1/2 and 10 patients with HAE-unknown) were collected. Of the patients with HAE-1/2, 53.4% experienced EM, whereas 43.1% did not. The forearm was the most frequent area of EM (64.5%), followed by the abdomen (29.0%) and upper arm and precordium (19.3%). Of the HAE-1/2 patients with EM, 41.9% always had angioedema following EM, while 29.0% always had colocalization of EM with angioedema. Moreover, 3.2% showed a correlation between the awareness of EM and severity of an angioedema attack. In 60.9% of HAE-1/2 patients with EM, the interval between the awareness of EM and appearance of angioedema was <3 h. Of the patients with HAE-unknown, 30.0% also experienced EM. Conclusion: We confirmed that more than one-half of Japanese patients with HAE-1/2 and one-third of those with HAE-unknown develop EM as the prodromal symptom of an angioedema attack. Physicians should communicate the significance of EM to patients with HAE to prepare them for possible imminent attacks.http://www.sciencedirect.com/science/article/pii/S1939455121000053Hereditary angioedemaProdromal symptomErythema marginatumBradykininC1-inhibitorIcatibant |