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Epidermolysis bullosa simplex

disorder
SNOMED 67144006CUI C0079298

Overview

Source: MedlinePlus.gov, National Library of Medicine. Not a substitute for medical advice.

Research Evidence

Peer-reviewed studies linked via MeSH term "Epidermolysis Bullosa Simplex" from the MEDLINE/PubMed database.

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Keratin-associated epidermolysis bullosa simplex: phenotypes and challenges in clinical trials - a narrative review and systematic update.
[object Object], [object Object], [object Object] et al. · Orphanet J Rare Dis · 2025
PMID: 40542406Meta-AnalysisFull text (PMC)
Pathogenesis and clinical features of alopecia in epidermolysis bullosa: A systematic review.
[object Object], [object Object], [object Object] et al. · Pediatr Dermatol · 2019
PMID: 31177584Meta-Analysis
Diacerein 1% Ointment for the Treatment of Epidermolysis Bullosa Simplex: A Randomized, Controlled Trial.
[object Object], [object Object], [object Object] et al. · J Drugs Dermatol · 2023
PMID: 37276163RCT
Topical diacerein for epidermolysis bullosa: a randomized controlled pilot study.
[object Object], [object Object], [object Object] et al. · Orphanet J Rare Dis · 2013
PMID: 23651789RCTFull text (PMC)
Epidermolysis Bullosa: Two rare case reports of COL7A1 and EBS-GEN SEV KRT14 variants with review of literature.
[object Object], [object Object], [object Object] et al. · BMC Pediatr · 2024
PMID: 38580989ReviewFull text (PMC)
Pathological Mechanisms Involved in Epidermolysis Bullosa Simplex: Current Knowledge and Therapeutic Perspectives.
[object Object], [object Object], [object Object] et al. · Int J Mol Sci · 2024
PMID: 39273442ReviewFull text (PMC)
Posttranslational modifications of keratins and their associated proteins as therapeutic targets in keratin diseases.
[object Object], [object Object], [object Object] et al. · Curr Opin Cell Biol · 2023
PMID: 37925932Review
Eye Involvement and Management in Inherited Epidermolysis Bullosa.
[object Object], [object Object], [object Object] et al. · Drugs · 2022
PMID: 36074321Review

Signs & Symptoms

Based on Human Phenotype Ontology (HPO) disease-phenotype annotations.

Cleavage within the basal keratinocyte layer
Always present (100%)HP:0034193
Foot pain
Very frequent (80-99%)HP:0025238
Hyperkeratosis, palmoplantar, focal friction-related
Very frequent (80-99%)HP:0007497
Junctional split
Very frequent (80-99%)HP:0003341
Palmoplantar blistering
Very frequent (80-99%)HP:0007446
Skin bullae
Very frequent (80-99%)HP:0008066
Skin fragility with non-scarring blistering
Very frequent (80-99%)HP:0007585
Skin itching
Very frequent (80-99%)HP:0000989
Acute episodes of neuropathic symptoms
Frequent (30-79%)HP:0003489
Intolerance to heat and fevers
Frequent (30-79%)HP:0002046
Paresthesia
Frequent (30-79%)HP:0003401
Skin plaque
Frequent (30-79%)HP:0200035
Upper limb pain
Frequent (30-79%)HP:0012513
Erosion of oral mucosa
Occasional (5-29%)HP:0031446
Palmar hyperkeratosis
Occasional (5-29%)HP:0010765
Plantar hyperkeratosis
Occasional (5-29%)HP:0007556
Profuse sweating
Occasional (5-29%)HP:0000975
Skin erosion
Occasional (5-29%)HP:0200041
Bullae of oral mucosa
Very rare (1-4%)HP:0200097
Dystrophic nails
Very rare (1-4%)HP:0008404
Erythematous papule
Very rare (1-4%)HP:0030350
Milia
Very rare (1-4%)HP:0001056
Thin, atrophic scars
Very rare (1-4%)HP:0001075
Epidermal hyperkeratosis
Excluded (<1%)HP:0000962

Quick Facts

SNOMED CT
67144006
UMLS CUI
C0079298
Fully Specified Name
Epidermolysis bullosa simplex (disorder)
Specialists
0
Diagnostic Biomarkers
0
HPO Phenotypes
24
Medical Disclaimer

This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider regarding any medical condition or treatment plan.

Clinical content is derived from the SNOMED CT clinical ontology and curated medical knowledge graphs.