Late-onset Morbus Hailey-Hailey

Marie-Eline P H Debeuf; Babette J A Verkouteren; Antoni H Gostynski; Myrurgia A Hamid; Michel Van Geel; Peter M Steijlen; Valerie L R M Verstraeten

doi:10.1684/ejd.2024.4657

Late-onset Morbus Hailey-Hailey

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Hailey-Hailey disease (HHD) is an autosomal dominant blistering disease, characterized by painful erythematous plaques with erosions, vesicles and fissures, predominantly affecting the skin folds. A pathogenic variant of the ATP2C1 gene, encoding the intracellular calcium/manganese pump, human secretory pathway Ca2+/Mn2+ ATPase (hSPCA1), leads to loss of intercellular connectivity between keratinocytes, i.e. acantholysis [1]. The disease typically presents within the third or fourth decade of life [...]

Original language	English
Pages (from-to)	218-219
Number of pages	2
Journal	European journal of dermatology : EJD
Volume	34
Issue number	2
DOIs	https://doi.org/10.1684/ejd.2024.4657
Publication status	Published - 1 Mar 2024

Keywords

Humans
Pemphigus, Benign Familial/pathology diagnosis
Female
Male
Age of Onset
Middle Aged

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10.1684/ejd.2024.4657

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title = "Late-onset Morbus Hailey-Hailey",

abstract = "Hailey-Hailey disease (HHD) is an autosomal dominant blistering disease, characterized by painful erythematous plaques with erosions, vesicles and fissures, predominantly affecting the skin folds. A pathogenic variant of the ATP2C1 gene, encoding the intracellular calcium/manganese pump, human secretory pathway Ca2+/Mn2+ ATPase (hSPCA1), leads to loss of intercellular connectivity between keratinocytes, i.e. acantholysis [1]. The disease typically presents within the third or fourth decade of life [...]",

keywords = "Humans, Pemphigus, Benign Familial/pathology diagnosis, Female, Male, Age of Onset, Middle Aged",

author = "Debeuf, \{Marie-Eline P H\} and Verkouteren, \{Babette J A\} and Gostynski, \{Antoni H\} and Hamid, \{Myrurgia A\} and \{Van Geel\}, Michel and Steijlen, \{Peter M\} and Verstraeten, \{Valerie L R M\}",

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doi = "10.1684/ejd.2024.4657",

language = "English",

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AU - Debeuf, Marie-Eline P H

AU - Verkouteren, Babette J A

AU - Gostynski, Antoni H

AU - Hamid, Myrurgia A

AU - Van Geel, Michel

AU - Steijlen, Peter M

AU - Verstraeten, Valerie L R M

PY - 2024/3/1

Y1 - 2024/3/1

N2 - Hailey-Hailey disease (HHD) is an autosomal dominant blistering disease, characterized by painful erythematous plaques with erosions, vesicles and fissures, predominantly affecting the skin folds. A pathogenic variant of the ATP2C1 gene, encoding the intracellular calcium/manganese pump, human secretory pathway Ca2+/Mn2+ ATPase (hSPCA1), leads to loss of intercellular connectivity between keratinocytes, i.e. acantholysis [1]. The disease typically presents within the third or fourth decade of life [...]

AB - Hailey-Hailey disease (HHD) is an autosomal dominant blistering disease, characterized by painful erythematous plaques with erosions, vesicles and fissures, predominantly affecting the skin folds. A pathogenic variant of the ATP2C1 gene, encoding the intracellular calcium/manganese pump, human secretory pathway Ca2+/Mn2+ ATPase (hSPCA1), leads to loss of intercellular connectivity between keratinocytes, i.e. acantholysis [1]. The disease typically presents within the third or fourth decade of life [...]

KW - Humans

KW - Pemphigus, Benign Familial/pathology diagnosis

KW - Female

KW - Male

KW - Age of Onset

KW - Middle Aged

U2 - 10.1684/ejd.2024.4657

DO - 10.1684/ejd.2024.4657

M3 - Article

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VL - 34

SP - 218

EP - 219

JO - European journal of dermatology : EJD

JF - European journal of dermatology : EJD

IS - 2

ER -

Late-onset Morbus Hailey-Hailey

Abstract

Keywords

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