Inherited epidermodysplasia verruciformis

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Inherited epidermodysplasia verruciformis

ORPHA:302Disease

Also called Lewandowsky-Lutz syndrome · Lutz-Lewandowsky epidermodysplasia verruciformis

What it is

Epidermodysplasia verruciformis (EV) is a rare inherited genodermatosis characterized by chronic infection with human papillomavirus (HPV) leading to polymorphous cutaneous lesions and high risk of developing non melanoma skin cancer.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
All ages
Inheritance
Autosomal recessive
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

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.

Genes

CIB1Disease-causing germline mutation(s)
IL7Disease-causing germline mutation(s)
TMC6Disease-causing germline mutation(s) (loss of function)
TMC8Disease-causing germline mutation(s) (loss of function)

ICD-10 codes

B07filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 6357MEDDRA 10052339MESH D004819MONDO 0009176OMIM 226400OMIM 305350OMIM 618231OMIM 618267OMIM 618309UMLS C0014522

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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