A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic is a rare, autoinflammatory disorder caused by a somatic mutation of ubiquitin-activating enzyme 1. Common systemic manifestations include fever, neutrophilic dermatoses, relapsing polychondritis, and pulmonary manifestations. Cutaneous manife...

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Main Authors: Anne-Sophie Groleau, Alex Derstenfeld, Steffany Poupart, Isabelle Marcil
Format: Article
Language:English
Published: SAGE Publishing 2025-05-01
Series:SAGE Open Medical Case Reports
Online Access:https://doi.org/10.1177/2050313X251341512
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author Anne-Sophie Groleau
Alex Derstenfeld
Steffany Poupart
Isabelle Marcil
author_facet Anne-Sophie Groleau
Alex Derstenfeld
Steffany Poupart
Isabelle Marcil
author_sort Anne-Sophie Groleau
collection DOAJ
description Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic is a rare, autoinflammatory disorder caused by a somatic mutation of ubiquitin-activating enzyme 1. Common systemic manifestations include fever, neutrophilic dermatoses, relapsing polychondritis, and pulmonary manifestations. Cutaneous manifestations are frequently the initial presentation and are a marker of systemic disease. Here, we present an unusual case of a 71-year-old man with multiple cutaneous manifestations of vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic syndrome occurring simultaneously.
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issn 2050-313X
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publishDate 2025-05-01
publisher SAGE Publishing
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series SAGE Open Medical Case Reports
spelling doaj-art-6ec77da5c23246efbc7b5c3dc92a55492025-08-20T03:08:22ZengSAGE PublishingSAGE Open Medical Case Reports2050-313X2025-05-011310.1177/2050313X251341512A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndromeAnne-Sophie Groleau0Alex Derstenfeld1Steffany Poupart2Isabelle Marcil3Division of Dermatology, Department of Medicine, Centre Hospitalier Universitaire de Montreal (CHUM), Université de Montréal, QC, CanadaDivision of Dermatology, Department of Medicine, Centre Hospitalier Universitaire de Montreal (CHUM), Université de Montréal, QC, CanadaDivision of Dermatology, Department of Medicine, Centre Hospitalier Universitaire de Montreal (CHUM), Université de Montréal, QC, CanadaDivision of Dermatology, Department of Medecine, Université de Montréal, QC, CanadaVacuoles, E1 enzyme, X-linked, autoinflammatory, somatic is a rare, autoinflammatory disorder caused by a somatic mutation of ubiquitin-activating enzyme 1. Common systemic manifestations include fever, neutrophilic dermatoses, relapsing polychondritis, and pulmonary manifestations. Cutaneous manifestations are frequently the initial presentation and are a marker of systemic disease. Here, we present an unusual case of a 71-year-old man with multiple cutaneous manifestations of vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic syndrome occurring simultaneously.https://doi.org/10.1177/2050313X251341512
spellingShingle Anne-Sophie Groleau
Alex Derstenfeld
Steffany Poupart
Isabelle Marcil
A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
SAGE Open Medical Case Reports
title A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
title_full A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
title_fullStr A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
title_full_unstemmed A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
title_short A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
title_sort series of cutaneous manifestations in a patient with vacuoles e1 enzyme x linked autoinflammatory somatic syndrome
url https://doi.org/10.1177/2050313X251341512
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