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2014 ; 107
(6
): 228-236
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Management of pyoderma gangrenosum
#MMPMID24912791
Teagle A
; Hargest R
J R Soc Med
2014[Jun]; 107
(6
): 228-236
PMID24912791
show ga
Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disease often associated
with underlying systemic diseases. Its pathogenesis is unknown, although immune
pathways have been implicated. Targeted therapy is therefore lacking and
currently treatment is largely empirical and consists of corticosteroids and
ciclosporin first line. This paper reviews the current and emerging knowledge
about PG. PG occurs with an incidence of 3-10 per million per year. The ulcers
are exquisitely painful and characteristically have a necrotic centre with
irregular overhanging bluish borders. Around half of cases are associated with
underlying systemic disease, most commonly inflammatory bowel disease, rheumatoid
arthritis and haematological malignancies; the remaining cases are idiopathic.
The pathogenesis is unknown, but the most widely supported theory is
immunological, and biopsies of lesions show a predominantly neutrophilic
infiltrate. Several aberrant immune processes have been described, with
neutrophils and their recruitment to sites of inflammation by cytokines taking an
apparently important role. Topical and systemic therapies are both vital aspects
of treatment, and in recent years, immune modulators have been used with
increasing success, with an emerging role for anti-tumour necrosis factor alpha
agents such as the monoclonal antibody infliximab. Although uncommon, PG causes
significant morbidity to those it affects. Further research is needed into the
disease pathogenesis, and adequate targeted treatment.