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Trait

Pyoderma gangrenosum

A deep skin ulcer with a well defined border, which is usually violet or blue. The ulcer edge is often undermined (worn and damaged) and the surrounding skin is erythematous and indurated. The ulcer often starts as a small papule or collection of papules, which break down to form small ulcers with a so called cat's paw appearance. These coalesce and the central area then undergoes necrosis to form a single ulcer.

HPO HP:0025452

Summary

Body system
Abnormality of the integument

11 genes linked to Pyoderma gangrenosum

Changes in these genes cause rare conditions in which Pyoderma gangrenosum is seen. Most people with this trait have none of those conditions, and a gene on this list says nothing about anyone's own genome.

GeneHow often it is seenConditions
PSTPIP1proline-serine-threonine phosphatase interacting protein 1AlwaysIn 1 condition
MEFVMEFV innate immunity regulator, pyrinVery often (80–99%)In 2 conditions
WDR1WD repeat domain 1Often (30–79%)In 1 condition
CLPBClpB family mitochondrial disaggregaseSometimes (5–29%)In 1 condition
ELANEelastase, neutrophil expressedSometimes (5–29%)In 1 condition
GFI1growth factor independent 1 transcriptional repressorSometimes (5–29%)In 1 condition
NFKB1nuclear factor kappa B subunit 1Sometimes (5–29%)In 1 condition
PTPN6protein tyrosine phosphatase non-receptor type 6Sometimes (5–29%)In 1 condition
SRP19signal recognition particle 19Sometimes (5–29%)In 1 condition
TCIRG1T cell immune regulator 1, ATPase H+ transporting V0 subunit a3Sometimes (5–29%)In 1 condition
OTULINOTU deubiquitinase with linear linkage specificityNot recordedIn 1 condition

How often is among people with one of the gene's conditions, the most often across them, as the Human Phenotype Ontology records it. These pages describe traits and the research behind them; they are not a diagnosis, and a doctor or genetic counsellor is the person to ask about symptoms.

Broader traits

Open skin sore

Where this comes from

  • The Human Phenotype Ontology, release 2026-09-01: the trait, its names, its definition and the genes linked to it. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

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