Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia

Abstract Background Hypohidrotic ectodermal dysplasia (HED) is a group of genodermatoses in which deficient ectodysplasin A signalling leads to maldevelopment of skin appendages, various eccrine glands, and teeth. Individuals with HED often have disrupted epithelial barriers and, therefore, were sus...

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Main Authors: Verena Hennig, Wolfgang Schuh, Antje Neubert, Dirk Mielenz, Hans-Martin Jäck, Holm Schneider
Format: Article
Language:English
Published: BMC 2021-09-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:https://doi.org/10.1186/s13023-021-02011-z
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spelling doaj-3f8f6c6aa7b54aaaba2c6936b52e35dd2021-09-05T11:35:24ZengBMCOrphanet Journal of Rare Diseases1750-11722021-09-011611910.1186/s13023-021-02011-zIncreased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasiaVerena Hennig0Wolfgang Schuh1Antje Neubert2Dirk Mielenz3Hans-Martin Jäck4Holm Schneider5Department of Pediatrics, University Hospital Erlangen, University of Erlangen-NürnbergDivision of Molecular Immunology, University Hospital Erlangen, University of Erlangen-NürnbergDepartment of Pediatrics, University Hospital Erlangen, University of Erlangen-NürnbergDivision of Molecular Immunology, University Hospital Erlangen, University of Erlangen-NürnbergDivision of Molecular Immunology, University Hospital Erlangen, University of Erlangen-NürnbergDepartment of Pediatrics, University Hospital Erlangen, University of Erlangen-NürnbergAbstract Background Hypohidrotic ectodermal dysplasia (HED) is a group of genodermatoses in which deficient ectodysplasin A signalling leads to maldevelopment of skin appendages, various eccrine glands, and teeth. Individuals with HED often have disrupted epithelial barriers and, therefore, were suspected to be more susceptible to coronavirus infection. Methods 56 households with at least one member who had coronavirus disease of 2019 (COVID-19) were enrolled in a longitudinal study to compare the course of illness, immune responses, and long-term consequences of severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection in HED patients (n = 15, age 9–52 years) and control subjects of the same age group (n = 149). Results In 14 HED patients, mild or moderate typical COVID-19 symptoms were observed that lasted for 4–45 days. Fever during the first days sometimes required external cooling measures. The course of COVID-19 was similar to that in control subjects if patients developed antibodies blocking the SARS-CoV-2 spike protein. Five out of six HED patients with completely abrogated ectodysplasin A signalling (83%) suffered from chronic, in two cases very severe fatigue following COVID-19, while only 25% of HED patients with residual activity of this pathway and 21% of control subjects recovering from COVID-19 experienced postinfectious fatigue. Hair loss after COVID-19 was also more frequent among HED patients (64%) than in the control group (13%). Conclusions HED appears to be associated with an increased risk of long-term consequences of a SARS-CoV-2 infection. Preventive vaccination against COVID-19 should be recommended for individuals affected by this rare genetic disorder.https://doi.org/10.1186/s13023-021-02011-zHypohidrotic ectodermal dysplasiaSARS-CoV-2COVID-19Chronic fatigueHair loss
collection DOAJ
language English
format Article
sources DOAJ
author Verena Hennig
Wolfgang Schuh
Antje Neubert
Dirk Mielenz
Hans-Martin Jäck
Holm Schneider
spellingShingle Verena Hennig
Wolfgang Schuh
Antje Neubert
Dirk Mielenz
Hans-Martin Jäck
Holm Schneider
Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
Orphanet Journal of Rare Diseases
Hypohidrotic ectodermal dysplasia
SARS-CoV-2
COVID-19
Chronic fatigue
Hair loss
author_facet Verena Hennig
Wolfgang Schuh
Antje Neubert
Dirk Mielenz
Hans-Martin Jäck
Holm Schneider
author_sort Verena Hennig
title Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
title_short Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
title_full Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
title_fullStr Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
title_full_unstemmed Increased risk of chronic fatigue and hair loss following COVID-19 in individuals with hypohidrotic ectodermal dysplasia
title_sort increased risk of chronic fatigue and hair loss following covid-19 in individuals with hypohidrotic ectodermal dysplasia
publisher BMC
series Orphanet Journal of Rare Diseases
issn 1750-1172
publishDate 2021-09-01
description Abstract Background Hypohidrotic ectodermal dysplasia (HED) is a group of genodermatoses in which deficient ectodysplasin A signalling leads to maldevelopment of skin appendages, various eccrine glands, and teeth. Individuals with HED often have disrupted epithelial barriers and, therefore, were suspected to be more susceptible to coronavirus infection. Methods 56 households with at least one member who had coronavirus disease of 2019 (COVID-19) were enrolled in a longitudinal study to compare the course of illness, immune responses, and long-term consequences of severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection in HED patients (n = 15, age 9–52 years) and control subjects of the same age group (n = 149). Results In 14 HED patients, mild or moderate typical COVID-19 symptoms were observed that lasted for 4–45 days. Fever during the first days sometimes required external cooling measures. The course of COVID-19 was similar to that in control subjects if patients developed antibodies blocking the SARS-CoV-2 spike protein. Five out of six HED patients with completely abrogated ectodysplasin A signalling (83%) suffered from chronic, in two cases very severe fatigue following COVID-19, while only 25% of HED patients with residual activity of this pathway and 21% of control subjects recovering from COVID-19 experienced postinfectious fatigue. Hair loss after COVID-19 was also more frequent among HED patients (64%) than in the control group (13%). Conclusions HED appears to be associated with an increased risk of long-term consequences of a SARS-CoV-2 infection. Preventive vaccination against COVID-19 should be recommended for individuals affected by this rare genetic disorder.
topic Hypohidrotic ectodermal dysplasia
SARS-CoV-2
COVID-19
Chronic fatigue
Hair loss
url https://doi.org/10.1186/s13023-021-02011-z
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