Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

doi:10.1186/s13023-020-01403-x

Review

. 2020 Jun 6;15(1):142.

doi: 10.1186/s13023-020-01403-x.

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Affiliations

¹ St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
² Dermatology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
³ Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
⁴ Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁵ Dermatology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
⁶ Pediatric Ophthalmology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
⁷ Paediatric Dermatology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁸ Endoscopy and Digestive Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁹ Department of Pain and Palliative Medicine, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
¹⁰ Department of Dermatology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Breisgau, Germany.
¹¹ Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France. ChristineBodemer@aphp.fr.
¹² Service de Dermatologie, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015, Paris, France. ChristineBodemer@aphp.fr.

PMID: 32505191
PMCID: PMC7276067
DOI: 10.1186/s13023-020-01403-x

Review

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Jemima E Mellerio et al. Orphanet J Rare Dis. 2020.

. 2020 Jun 6;15(1):142.

doi: 10.1186/s13023-020-01403-x.

Authors

Affiliations

¹ St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
² Dermatology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
³ Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
⁴ Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁵ Dermatology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
⁶ Pediatric Ophthalmology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
⁷ Paediatric Dermatology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁸ Endoscopy and Digestive Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁹ Department of Pain and Palliative Medicine, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
¹⁰ Department of Dermatology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Breisgau, Germany.
¹¹ Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France. ChristineBodemer@aphp.fr.
¹² Service de Dermatologie, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015, Paris, France. ChristineBodemer@aphp.fr.

PMID: 32505191
PMCID: PMC7276067
DOI: 10.1186/s13023-020-01403-x

Abstract

Epidermolysis bullosa (EB) comprises a group of genetic disorders with the hallmark of fragility of the skin and mucosal surfaces. The severity of different types of EB varies markedly as does the occurrence of extra-cutaneous involvement and complications. A number of emergency situations may occur in the context of EB including obstruction to oral intake from oral or esophageal blisters or scarring, acute airway obstruction, acute urinary retention, sepsis and corneal erosions. Whilst general management principles apply in each of these settings, specific considerations are essential in managing EB to avoid undue trauma or damage to delicate tissues. These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.

Keywords: Airway obstruction; Blister; Corneal erosion; ERN-skin; Epidermolysis bullosa; Esophageal obstruction; Pain; Sepsis; Urinary retention.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

**Fig. 1**
Emergency card for EB patients. In English, French, German and Italian

See this image and copyright information in PMC

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References

1. Fine JD, Johnson LB, Weiner M, Suchindran C. Cause-specific risks of childhood death in inherited epidermolysis bullosa. J Pediatr. 2008;152:276–280. doi: 10.1016/j.jpeds.2007.06.039. - DOI - PubMed
1. Kho YC, Rhodes LM, Robertson SJ, et al. Epidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa registry with a focus on Herlitz junctional epidermolysis bullosa. Arch Dermatol. 2010;146:635–640. doi: 10.1001/archdermatol.2010.109. - DOI - PubMed
1. Yuen WY, Duipmans JC, Molenbuur B, et al. Long-term follow-up of patients with Herlitz-type junctional epidermolysis bullosa. Br J Dermatol. 2012;167:374–382. doi: 10.1111/j.1365-2133.2012.10997.x. - DOI - PubMed
1. Fine JD, Johnson LB, Weiner M, Suchindran C. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastrolenterol Nutr. 2008;46:147–158. doi: 10.1097/MPG.0b013e31812f5667. - DOI - PubMed
1. El Hachem M, Zambruno G, Bourdon-Lanoy E, et al. Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Orphanet J Rare Dis. 2014;20:76. doi: 10.1186/1750-1172-9-76. - DOI - PMC - PubMed

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[1] Fine JD, Johnson LB, Weiner M, Suchindran C. Cause-specific risks of childhood death in inherited epidermolysis bullosa. J Pediatr. 2008;152:276–280. doi: 10.1016/j.jpeds.2007.06.039. - DOI - PubMed

[2] Fine JD, Johnson LB, Weiner M, Suchindran C. Cause-specific risks of childhood death in inherited epidermolysis bullosa. J Pediatr. 2008;152:276–280. doi: 10.1016/j.jpeds.2007.06.039. - DOI - PubMed

[3] Kho YC, Rhodes LM, Robertson SJ, et al. Epidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa registry with a focus on Herlitz junctional epidermolysis bullosa. Arch Dermatol. 2010;146:635–640. doi: 10.1001/archdermatol.2010.109. - DOI - PubMed

[4] Kho YC, Rhodes LM, Robertson SJ, et al. Epidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa registry with a focus on Herlitz junctional epidermolysis bullosa. Arch Dermatol. 2010;146:635–640. doi: 10.1001/archdermatol.2010.109. - DOI - PubMed

[5] Yuen WY, Duipmans JC, Molenbuur B, et al. Long-term follow-up of patients with Herlitz-type junctional epidermolysis bullosa. Br J Dermatol. 2012;167:374–382. doi: 10.1111/j.1365-2133.2012.10997.x. - DOI - PubMed

[6] Yuen WY, Duipmans JC, Molenbuur B, et al. Long-term follow-up of patients with Herlitz-type junctional epidermolysis bullosa. Br J Dermatol. 2012;167:374–382. doi: 10.1111/j.1365-2133.2012.10997.x. - DOI - PubMed

[7] Fine JD, Johnson LB, Weiner M, Suchindran C. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastrolenterol Nutr. 2008;46:147–158. doi: 10.1097/MPG.0b013e31812f5667. - DOI - PubMed

[8] Fine JD, Johnson LB, Weiner M, Suchindran C. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastrolenterol Nutr. 2008;46:147–158. doi: 10.1097/MPG.0b013e31812f5667. - DOI - PubMed

[9] El Hachem M, Zambruno G, Bourdon-Lanoy E, et al. Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Orphanet J Rare Dis. 2014;20:76. doi: 10.1186/1750-1172-9-76. - DOI - PMC - PubMed

[10] El Hachem M, Zambruno G, Bourdon-Lanoy E, et al. Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Orphanet J Rare Dis. 2014;20:76. doi: 10.1186/1750-1172-9-76. - DOI - PMC - PubMed

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Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Affiliations

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

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References

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Abstract

Conflict of interest statement

Figures

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Cited by

References

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