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Inherited epidermolysis bullosa and squamous cell carcinoma: a systematic review of 117 cases

Overview of attention for article published in Orphanet Journal of Rare Diseases, January 2016
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  • Above-average Attention Score compared to outputs of the same age (59th percentile)
  • Good Attention Score compared to outputs of the same age and source (77th percentile)

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4 tweeters
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3 Facebook pages

Citations

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50 Dimensions

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61 Mendeley
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Title
Inherited epidermolysis bullosa and squamous cell carcinoma: a systematic review of 117 cases
Published in
Orphanet Journal of Rare Diseases, January 2016
DOI 10.1186/s13023-016-0489-9
Pubmed ID
Authors

H. Montaudié, C. Chiaverini, E. Sbidian, A. Charlesworth, J-P. Lacour, Montaudié, H, Chiaverini, C, Sbidian, E, Charlesworth, A, Lacour, J-P

Abstract

Inherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by exacerbated skin and/or mucosal fragility and blister formation after minor mechanical trauma. Level of cleavage in the skin, clinical features with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy and/or gene involved, type(s) of mutation present and sometimes specific mutation(s), allow to define the EB type and subtype. This family of genodermatoses exposes patients to several complications, cutaneous squamous cell carcinoma (cSCC) being the most severe of them. The aim of this systematic review was to document patients with EB who developed cSCC. A systematic literature search was performed, from inception to March 2014, using Medline, Embase, Cochrane and ClinicalTrials.gov databases. Only articles published in English and French were selected. The diagnosis of EB had to be confirmed by EM and/or IFM and/or mutation analysis, while cSCC had to be confirmed by histological analysis. Of 167 references in the original search, 69 relevant articles were identified, representing 117 cases. cSCCs were identified in all types of EB, though predominantly in recessive dystrophic EB (RDEB) forms (81 cases (69.2 %)). The median age at diagnosis was 36 years old (interquartile range (IQR), 27-48 years and range, 6-71 years) for all forms. Of those with measurements in the literature (88 cases (75.2 %)), tumor size was greater than 2 centimeters in 52 cases (59.1 %). The histopathological characteristics were specified in 88 cases (75.2 %) and well-differentiated forms predominated (73.9 %). No conclusion could be drawn on the choice of surgical treatment or the management in advanced forms. This study was retrospective and statistical analysis was not included due to various biases. This study design did not allow to infer prevalence, nor EB subtype risk for cSCC occurrence. Our study correlated with historical data shows that most of the cSCCs occurred in subjects with the RDEB subtype, however reports also show that cSCCs can present in any patients with EB. The first signs of cSCC developed at a younger age in EB patients than in non-EB patients. Interestingly, the cSCC duration, before its diagnosis, was shorter in individuals with RDEB than with junctional EB (JEB) and dominant dystrophic EB (DDEB). This study further emphasizes the importance of regular monitoring of EB patients, particularly with the RDEB subtype as they developed cSCC at a younger age.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 61 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Spain 1 2%
Unknown 60 98%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 13 21%
Researcher 8 13%
Student > Master 8 13%
Other 6 10%
Student > Doctoral Student 5 8%
Other 12 20%
Unknown 9 15%
Readers by discipline Count As %
Medicine and Dentistry 27 44%
Biochemistry, Genetics and Molecular Biology 13 21%
Agricultural and Biological Sciences 5 8%
Psychology 2 3%
Nursing and Health Professions 1 2%
Other 0 0%
Unknown 13 21%

Attention Score in Context

This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 01 September 2016.
All research outputs
#8,642,966
of 16,576,001 outputs
Outputs from Orphanet Journal of Rare Diseases
#809
of 1,760 outputs
Outputs of similar age
#105,734
of 268,215 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#5
of 22 outputs
Altmetric has tracked 16,576,001 research outputs across all sources so far. This one is in the 47th percentile – i.e., 47% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,760 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.4. This one has gotten more attention than average, scoring higher than 52% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 268,215 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 59% of its contemporaries.
We're also able to compare this research output to 22 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 77% of its contemporaries.