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Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients

Identifieur interne : 000E11 ( Istex/Corpus ); précédent : 000E10; suivant : 000E12

Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients

Auteurs : C. Hotz ; L. Valeyrie-Allanore ; C. Haddad ; S. Bouvresse ; N. Ortonne ; T. A. Duong ; S. Ingen-Housz-Oro ; J. C. Roujeau ; P. Wolkenstein ; O. Chosidow

Source :

RBID : ISTEX:A841FBAA7052D19B28CD087BAE99FC17670D964A

English descriptors

Abstract

Background: Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts. Objectives: To investigate the frequency and clinical features of AGEP systemic involvement. Methods: This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work‐up available. Results: Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C‐reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone‐marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments. Conclusions: The neutrophil count–systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.
What's already known about this topic? Internal organ dysfunction is not usually expected in acute generalized exanthematous pustulosis (AGEP). Systemic involvement in AGEP has rarely been reported. What does this study add? AGEP systemic involvement does exist. Increased physician awareness and better knowledge of the condition should improve its management.

Url:
DOI: 10.1111/bjd.12502

Links to Exploration step

ISTEX:A841FBAA7052D19B28CD087BAE99FC17670D964A

Le document en format XML

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<term>Acute</term>
<term>Agep</term>
<term>Alanine aminotransferase</term>
<term>Amoxicillin</term>
<term>Amoxicillin rechallenge</term>
<term>Arch dermatol</term>
<term>Aspartate aminotransferase</term>
<term>Blood gases</term>
<term>Body surface area</term>
<term>British association</term>
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<term>Bronchoalveolar lavage</term>
<term>Cholestasis</term>
<term>Creatinine</term>
<term>Cutaneous</term>
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<term>Dermatologist</term>
<term>Dermatology</term>
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<term>Distress syndrome</term>
<term>Drug reactions</term>
<term>Euroscar</term>
<term>Euroscar score</term>
<term>Exanthematous</term>
<term>Exanthematous pustulosis</term>
<term>Generalized pustular psoriasis</term>
<term>Hepatic</term>
<term>Hepatic dysfunction</term>
<term>Hepatic function tests</term>
<term>Hepatocellular</term>
<term>High fever</term>
<term>Hotz</term>
<term>Involvement</term>
<term>Liver cholestasis</term>
<term>Neutrophil</term>
<term>Neutrophil count</term>
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<term>Pustule</term>
<term>Pustulosis</term>
<term>Putative culprit drugs</term>
<term>Rechallenge</term>
<term>Renal</term>
<term>Renal function tests</term>
<term>Retrospective study</term>
<term>Severe cutaneous</term>
<term>Severe cutaneous involvement</term>
<term>Syndrome</term>
<term>Systemic</term>
<term>Systemic involvement</term>
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<div type="abstract">Background: Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts. Objectives: To investigate the frequency and clinical features of AGEP systemic involvement. Methods: This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work‐up available. Results: Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C‐reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone‐marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments. Conclusions: The neutrophil count–systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.</div>
<div type="abstract" xml:lang="en">What's already known about this topic? Internal organ dysfunction is not usually expected in acute generalized exanthematous pustulosis (AGEP). Systemic involvement in AGEP has rarely been reported. What does this study add? AGEP systemic involvement does exist. Increased physician awareness and better knowledge of the condition should improve its management.</div>
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<head>Summary</head>
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<p>Acute generalized exanthematous pustulosis (
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) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts.</p>
<head>Objectives</head>
<p>To investigate the frequency and clinical features of
<hi rend="fc">AGEP</hi>
systemic involvement.</p>
<head>Methods</head>
<p>This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of
<hi rend="fc">AGEP</hi>
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<hi rend="fc">SCAR</hi>
score > 4 and (ii) biological and radiological work‐up available.</p>
<head>Results</head>
<p>Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C‐reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement.
<hi rend="fc">AGEP</hi>
systemic involvement was observed in 17% of cases studied, including liver, kidney, bone‐marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments.</p>
<head>Conclusions</head>
<p>The neutrophil count–systemic involvement association may suggest a role for neutrophils in
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systemic involvement. Physicians should be aware of the possibility of systemic involvement in
<hi rend="fc">AGEP</hi>
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<line>
<b>Correspondence</b>
</line>
<line>
<i>Laurence Valeyrie‐Allanore</i>
.</line>
<line>
<i>E‐mail:</i>
<email>laurence.allanore@hmn.aphp.fr</email>
</line>
</lineatedText>
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<link type="toTypesetVersion" href="file:BJD.BJD12502.pdf"></link>
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<title type="main">Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients</title>
<title type="shortAuthors">C. Hotz
<i>et al</i>
.</title>
</titleGroup>
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<familyName>Haddad</familyName>
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<personName>
<givenNames>S.</givenNames>
<familyName>Bouvresse</familyName>
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<personName>
<givenNames>N.</givenNames>
<familyName>Ortonne</familyName>
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<personName>
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<familyName>Duong</familyName>
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<personName>
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<personName>
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<familyName>Chosidow</familyName>
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<orgDiv>Department of Dermatology</orgDiv>
<orgName>Referral Center for Toxic and Autoimmune Blistering Diseases</orgName>
<orgName>Université–Paris‐Est Créteil Val de Marne (UPEC)</orgName>
<orgName>Henri‐Mondor Hospital</orgName>
<address>
<street>51, avenue du Maréchal de Tassigny</street>
<city>Créteil CEDEX</city>
<postCode>94010</postCode>
<country>France</country>
</address>
</affiliation>
<affiliation countryCode="FR" type="organization" xml:id="bjd12502-aff-0002">
<orgName>LIC EA 4393</orgName>
<orgDiv>Assistance Publique–Hôpitaux de Paris (APHP)</orgDiv>
<orgName>Université–Paris‐Est Créteil Val de Marne (UPEC)</orgName>
<orgName>Henri‐Mondor Hospital</orgName>
<address>
<street>51, avenue du Maréchal de Tassigny</street>
<city>Créteil CEDEX</city>
<postCode>94010</postCode>
<country>France</country>
</address>
</affiliation>
<affiliation countryCode="FR" type="organization" xml:id="bjd12502-aff-0003">
<orgDiv>Department of Pathology</orgDiv>
<orgDiv>Assistance Publique – Hôpitaux de Paris (APHP)</orgDiv>
<orgName>Université – Paris‐Est Créteil Val de Marne (UPEC)</orgName>
<orgName>Henri‐Mondor Hospital</orgName>
<address>
<street>51, avenue du Maréchal de Tassigny</street>
<city>Créteil CEDEX</city>
<postCode>94010</postCode>
<country>France</country>
</address>
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<affiliation countryCode="FR" type="organization" xml:id="bjd12502-aff-0004">
<orgName>INSERM</orgName>
<orgName>Centre d'Investigation Clinique 006</orgName>
<orgName>APHP</orgName>
<address>
<city>Créteil</city>
<postCode>F‐94000</postCode>
<country>France</country>
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<title type="main">Summary</title>
<section xml:id="bjd12502-sec-0001">
<title type="main">Background</title>
<p>Acute generalized exanthematous pustulosis (
<fc>AGEP</fc>
) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts.</p>
</section>
<section xml:id="bjd12502-sec-0002">
<title type="main">Objectives</title>
<p>To investigate the frequency and clinical features of
<fc>AGEP</fc>
systemic involvement.</p>
</section>
<section xml:id="bjd12502-sec-0003">
<title type="main">Methods</title>
<p>This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of
<fc>AGEP</fc>
. Patients had to fulfil the following criteria: (i) a specific Euro
<fc>SCAR</fc>
score > 4 and (ii) biological and radiological work‐up available.</p>
</section>
<section xml:id="bjd12502-sec-0004">
<title type="main">Results</title>
<p>Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C‐reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement.
<fc>AGEP</fc>
systemic involvement was observed in 17% of cases studied, including liver, kidney, bone‐marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments.</p>
</section>
<section xml:id="bjd12502-sec-0005">
<title type="main">Conclusions</title>
<p>The neutrophil count–systemic involvement association may suggest a role for neutrophils in
<fc>AGEP</fc>
systemic involvement. Physicians should be aware of the possibility of systemic involvement in
<fc>AGEP</fc>
and should actively look for signs of extracutaneous reactions.</p>
</section>
</abstract>
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<p>
<b>What's already known about this topic?</b>
</p>
<p>
<list formatted="paragraph" style="bulleted" xml:id="bjd12502-list-0001">
<listItem>Internal organ dysfunction is not usually expected in acute generalized exanthematous pustulosis (AGEP).</listItem>
<listItem>Systemic involvement in AGEP has rarely been reported.</listItem>
</list>
</p>
<p>
<b>What does this study add?</b>
</p>
<p>
<list formatted="paragraph" style="bulleted" xml:id="bjd12502-list-0002">
<listItem>AGEP systemic involvement does exist.</listItem>
<listItem>Increased physician awareness and better knowledge of the condition should improve its management.</listItem>
</list>
</p>
</abstract>
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<note numbered="no" xml:id="bjd12502-note-0001">
<b>Funding sources</b>
None.</note>
<note numbered="no" xml:id="bjd12502-note-0002">
<b>Conflicts of interest</b>
None declared.</note>
<note numbered="no" xml:id="bjd12502-note-0003">P.W. and O.C. contributed equally to this work.</note>
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<abstract>Background: Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts. Objectives: To investigate the frequency and clinical features of AGEP systemic involvement. Methods: This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work‐up available. Results: Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C‐reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone‐marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments. Conclusions: The neutrophil count–systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.</abstract>
<abstract type="short" lang="en">What's already known about this topic? Internal organ dysfunction is not usually expected in acute generalized exanthematous pustulosis (AGEP). Systemic involvement in AGEP has rarely been reported. What does this study add? AGEP systemic involvement does exist. Increased physician awareness and better knowledge of the condition should improve its management.</abstract>
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