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Start free with EleplanDystrophic epidermolysis bullosa pruriginosa
ORPHA:89843Disease
Also called DEB pruriginosa · DEB-Pr · Pruriginous dystrophic epidermolysis bullosa
What it is
A rare dystrophic epidermolysis bullosa (DEB) characterized by generalized or localized skin lesions associated with severe, if not intractable, pruritus.
Key facts
- Age of onset
- Childhood
- Inheritance
- Autosomal dominant, Autosomal recessive
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (Europe)Dystrophic epidermolysis bullosa
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Common30–79%
6Sometimes5–29%
11- Abnormality of head or neck
- Abnormality of the forearm
- Abnormal toenail morphology
- Atrophic scars
- Dermal atrophy
- Hyperkeratosis
- Junctional split
- Milia
and 3 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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