Acute generalized exanthematous pustulosis and polyarthritis associated with a novel CARD14 mutation

Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyar...

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Bibliographic Details
Published inAustralasian journal of dermatology Vol. 59; no. 1; pp. e70 - e73
Main Authors Podlipnik, Sebastian, Castellanos‐Moreira, Raul, Florez‐Enrich, Helena, Arostegui, Juan Ignacio, Mascaró, José Manuel
Format Journal Article
LanguageEnglish
Published Australia Wiley Subscription Services, Inc 01.02.2018
Subjects
acute generalised exanthematous pustulosis
AGEP
autoinflammatory
CARD14
Exanthema
Fever
Mutation
Pityriasis
Polyarthritis
Psoriasis
Pustulosis
toxicoderma
acute generalised exanthematous pustulosis
mutation
toxicoderma
autoinflammatory
CARD14
AGEP
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Summary:Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyarthritis, in which a novel CARD14 mutation was identified. The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients. Similarly, mutations in the CARD14 gene have been linked to pustular types of psoriasis and familiar cases of pityriasis rubra pilaris; however, there are no reports associating mutations in the CARD14 gene with AGEP.
ISSN:0004-8380
1440-0960
DOI:10.1111/ajd.12669