PRIMEVIEW

PYODERMA GANGRENOSUM
The main pathogenetic mechanism of PG For the Primer, visit doi:10.1038/s41572-020-0213-x
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immune system causes tissue damage in EPIDEMIOLOGY
Pyoderma gangrenosum (PG) is a rare
PATHOPHYSIOLOGY the absence of an external stimulus
neutrophilic dermatosis characterized
by painful non-infectious ulcers that Data on the prevalence of PG are scarce, and
develop rapidly, typically at sites of even estimates might be hindered by the difficulty in
minor trauma. PG often occurs with accurately diagnosing PG. PG affects adults and
other autoimmune and autoinflammatory elderly individuals most frequently but can occur at
diseases, such as inflammatory bowel Inflammasomes (WNNVJKEMPGUUWNEGTCVKQP any age. Although the percentage varies between
are cytosolic studies, a substantial proportion of individuals with
disease (IBD) and rheumatoid arthritis.
multiprotein complexes
PG also have another autoinflammatory disease or
that cleave and thereby 2GTKRJGTCNGT[VJGOC a malignancy, usually a haematological malignancy.
activate precursors of
DIAGNOSIS pro-nflammatory
cytokines, such as
PG lesions have undermined borders surrounded by IL-1β|CPF+.
-GTCVKPQE[VG QUALITY OF LIFE
erythema and, if untreated, quickly expand. PG can
be difficult to diagnose. A tissue biopsy is generally 4GFFKUJ
required and laboratory tests can also be helpful XKQNCEGQWU Quality of life is severely reduced in
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to rule out several differential diagnoses, such as patients with PG, mainly owing to pain.
DQTFGT inflammasomes
infections, vasculitis or autoimmune diseases. The appearance
is increased in PG;
of the lesions and
0GWVTQRJKN these complexes contain
Most PG ulcers caspase 1, which is the concerns over
occur on the enzyme that cleaves worsening or
NQYGTNGIURGTJCRU pro-inflammatory recurrent disease
because this area cytokine can also lead to
KUQȎGPGZRQUGFVQ precursors psychological
accidental trauma issues; for example,
depression occurs
in 10% or more
The of individuals
increased with PG.
MANAGEMENT
expression of
genes encoding
Treatment aims at reducing inflammation with pro-inflammatory
fast-acting systemic immunosuppressive drugs, cytokines in PG drives the OUTLOOK
usually a corticosteroid and/or cyclosporine. recruitment of immune
Other compatible drugs, including steroid- cells, particularly
sparing agents and biologics, can be added Adaptive Future research should address various gaps in the
neutrophils T cell
immunity
to the therapy if complete response is not PG field. Validated outcome measures are required
might also have a
achieved or in severe disease. Comorbidities to accurately assess PG severity and the results
role in PG, as T cells,
need to be considered when choosing the of clinical trials. In turn, sufficiently powered
type 1 T helper (TH1) cells
therapy; for example, in individuals with IBD, trials are needed to determine which immune
and TH17 cells have been
a biologic (such as an anti-TNF agent) could be detected in the immune pathway is the best therapeutic target. PG-specific
PG can manifest as part of other
a reasonable first option. Appropriate wound infiltrate of PG biomarkers would be useful for diagnosis and
neutrophilic dermatosis syndromic
care with gentle cleansing and dressing is lesions to predict disease course and responsiveness to
diseases, caused by mutations in genes
CNUQ|GUUGPVKCN specific therapies.
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doi:10.1038/s41572-020-00221-6; Article citation ID: (2020) 6:80 Written by Lucia Brunello; designed by Laura Marshall
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