ArticleThe British journal of dermatology2008
The risk of cardiomyopathy in inherited epidermolysis bullosa.
Article in The British journal of dermatology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 2 of them syntheses that pooled it.
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Who cites it
15 citing papers in PubMed, 2 syntheses or guidelines pooled it, 62 citations in OpenAlex.
- A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa.Orphanet journal of rare diseases · 2021Pooled it
- Pain care for patients with epidermolysis bullosa: best care practice guidelines.BMC medicine · 2014Guideline
- Amelioration of Systemic Amyloidosis by Blocking IL-17A and Not by IL-17F, and Arteriosclerosis by Blocking Both IL-17A and IL-17F in an Inflammatory Skin Mouse Model.International journal of molecular sciences · 2024Article
- Heterozygous nonsense variants in laminin subunit 3α resulting in Ebstein's anomaly.HGG advances · 2023Article
- Arteriosclerosis Derived from Cutaneous Inflammation Is Ameliorated by the Deletion of IL-17A and IL-17F.International journal of molecular sciences · 2023Article
- Dystrophic Epidermolysis Bullosa: Secondary Disease Mechanisms and Disease Modifiers.Frontiers in genetics · 2021Review
- The analysis of echocardiographic results in patients suffering from epidermolysis bullosa.Postepy dermatologii i alergologii · 2020Article
- Epidermolysis bullosa.Nature reviews. Disease primers · 2020Review
- Emaciation, Congestive Heart Failure, and Systemic Amyloidosis in Severe Recessive Dystrophic Epidermolysis Bullosa: Possible Internal Complications Due to Skin-Derived Inflammatory Cytokines Derived from the Injured Skin.Dermatopathology (Basel, Switzerland) · 2020Article
- Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa.Medicine · 2015Observational
- Dystrophic epidermolysis bullosa: a review.Clinical, cosmetic and investigational dermatology · 2015Review
- Article
- Inherited epidermolysis bullosa.Orphanet journal of rare diseases · 2010Review
- New Onset Mitral Regurgitation Caused by Mitral Valve Prolapse in a Male Patient with Dominant Pretibial Dystrophic Epidermolysis Bullosa.Indian journal of dermatologyArticle
- Hereditary epidermolysis bullosa: clinical-epidemiological profile of 278 patients at a tertiary hospital in São Paulo, Brazil.Anais brasileiros de dermatologiaObservational
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
backgroundCase reports have suggested that cardiomyopathy may be a complication of recessive dystrophic epidermolysis bullosa (RDEB).
objectiveTo determine the risk of congestive heart failure (CHF) or cardiomyopathy in each major EB subtype.
methodsThese data represent systematic case findings and data collection performed throughout the continental United States from 1986 through 2002, by the National Epidermolysis Bullosa Registry. Study design is cross-sectional (n = 3280) with a nested randomly sampled longitudinal subcohort (n = 450). Frequencies of CHF and cardiomyopathy were determined by patient self-reporting, medical histories and review of medical records. In those who died, death certificates were reviewed and histories obtained from surviving family. Cumulative risks were stratified by cause and EB subtype.
resultsCardiomyopathy was reported as early as within the first year of life. In patients having no other known risk factors for CHF or cardiomyopathy, the highest risk of cardiomyopathy was seen among patients with Hallopeau-Siemens RDEB (RDEB-HS), with a cumulative risk of 4.51% on or after age 20 years. The cumulative risk of cardiomyopathy was only 1.14% and 0.40% in non-Herlitz junctional EB (JEB) and non-Hallopeau-Siemens RDEB, respectively, and was not observed in any other EB subtype. When patients with coexistent chronic renal failure were included, the cumulative risk for RDEB-HS rose to 18.86% by age 35 years. About 30% of our patients affected with RDEB-HS died of CHF or cardiomyopathy, even those with no other known risk factors.
conclusionsCHF and cardiomyopathy are uncommon complications in both major RDEB subtypes and non-Herlitz JEB, and may be fatal.
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Registered trials
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