epidermolysis bullosa simplex with muscular dystrophy

autosomal recessive disease characterized early childhood onset of progressive muscular dystrophy and blistering skin changes and that has material basis in homozygous or compound heterozygous mutation in the PLEC gene on chromosome 8q24
MedicalCondition developmental_defect_during_embryogenesis Q5382849
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epidermolysis bullosa simplex with muscular dystrophy

Summary

epidermolysis bullosa simplex with muscular dystrophy is a developmental defect during embryogenesis[1].

Key Facts

  • epidermolysis bullosa simplex with muscular dystrophy's instance of is recorded as developmental defect during embryogenesis[2].
  • epidermolysis bullosa simplex with muscular dystrophy's instance of is recorded as class of disease[3].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of epidermolysis bullosa simplex[4].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of autosomal recessive disease[5].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of malformation syndrome with skin/mucosae involvement[6].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of hereditary epidermolysis bullosa associated with ocular features[7].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of basal epidermolysis bullosa simplex[8].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of qualitative or quantitative defects of plectin[9].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of autosomal recessive limb-girdle muscular dystrophy[10].
  • epidermolysis bullosa simplex with muscular dystrophy is a type of syndrome[11].
  • epidermolysis bullosa simplex with muscular dystrophy's health specialty is recorded as medical genetics[12].
  • epidermolysis bullosa simplex with muscular dystrophy's genetic association is recorded as PLEC[13].
  • epidermolysis bullosa simplex with muscular dystrophy's exact match is recorded as http://purl.obolibrary.org/obo/DOID_0090017[14].
  • epidermolysis bullosa simplex with muscular dystrophy's exact match is recorded as http://identifiers.org/doid/DOID:0090017[15].
  • epidermolysis bullosa simplex with muscular dystrophy's exact match is recorded as http://www.orpha.net/ORDO/Orphanet_257[16].
  • epidermolysis bullosa simplex with muscular dystrophy's on focus list of Wikimedia project is recorded as WikiProject Medicine[17].

References

Programmatic citations — every numbered marker resolves to a verifiable graph row below.

Direct Wikidata claims

  1. [2] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  2. [3] . wikidata.org.
  3. [4] . Downstream effects of plectin mutations in epidermolysis bullosa simplex with muscular dystrophy. wikidata.org.
  4. [5] . Disease Ontology. Retrieved . wikidata.org.
  5. [6] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  6. [7] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  7. [8] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  8. [9] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  9. [10] . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  10. [11] . Disease Ontology. Retrieved . wikidata.org.
  11. [12] . wikidata.org.
  12. [13] . Q905695. Retrieved . platform.opentargets.org. Provenance: wikidata.org.
  13. [14] . Disease Ontology. Retrieved . wikidata.org.
  14. [15] . Identifiers.org. registry.identifiers.org. Provenance: wikidata.org.
  15. [16] . wikidata.org.
  16. [17] . wikidata.org.

Class ancestry

  1. [1] . Wikidata. wikidata.org.

📑 Cite this page

Use these citations when quoting this entity in research, articles, AI prompts, or wherever provenance matters. We aggregate Wikidata + Wikipedia + authoritative open-data sources; the stitched, scored, cross-referenced view is what 4ort.xyz contributes.

APA 4ort.xyz Knowledge Graph. (2026). epidermolysis bullosa simplex with muscular dystrophy. Retrieved May 3, 2026, from https://4ort.xyz/entity/epidermolysis-bullosa-simplex-with-muscular-dystrophy
MLA “epidermolysis bullosa simplex with muscular dystrophy.” 4ort.xyz Knowledge Graph, 4ort.xyz, 3 May. 2026, https://4ort.xyz/entity/epidermolysis-bullosa-simplex-with-muscular-dystrophy.
BibTeX @misc{4ortxyz_epidermolysis-bullosa-simplex-with-muscular-dystrophy_2026, author = {{4ort.xyz Knowledge Graph}}, title = {{epidermolysis bullosa simplex with muscular dystrophy}}, year = {2026}, url = {https://4ort.xyz/entity/epidermolysis-bullosa-simplex-with-muscular-dystrophy}, note = {Accessed: 2026-05-03}}
LLM prompt According to 4ort.xyz Knowledge Graph (aggregator of Wikidata, Wikipedia, and authoritative open-data sources): epidermolysis bullosa simplex with muscular dystrophy — https://4ort.xyz/entity/epidermolysis-bullosa-simplex-with-muscular-dystrophy (retrieved 2026-05-03)

Canonical URL: https://4ort.xyz/entity/epidermolysis-bullosa-simplex-with-muscular-dystrophy · Last refreshed:

Edit History

Rolling log of changes to this entity's Wikidata record. Values shown reflect the current state of each edited property — follow the history link to see the precise diff for any edit.

  1. 1d ago · JhealdBatch bot · 2026-07-03 view diff on Wikidata ↗
    Mondo id MONDO_0009181
    Genetic association PLEC
    Gard rare disease id 2137
    Orphanet id 257
    + 13 other properties edited (see Wikidata diff for full list)
    "/* wbeditentity-update:0| */ QuickStatements 3.0 [[:toollabs:qs-dev/batch/39742|batch #39742]]: subclass of disease, not instance"
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