International Journal of Clinical Case Reports 2015, Vol.5, No. 50, 1-3
3
pyoderma gangrenosum and acne), a rare clinical
subtype of PG, which a hereditary, autosomal
dominant, auto-inflammatory disease caused by
mutations in the PSTPIP1 gene. The prol ine
-serine-threonine phosphatase interacting protein 1 is
involved in immune regulation (Zeeli et al.,
2015). Likewise, genetic studies on PG associated
with crohn’s disease have suggested association with
PSTPIP1, PTPN6, and TRAF3IP2 genes (Weizman et
al., 2014).
If left untreated, PG may last for months to years. Due
to the lack of randomized controlled trials, treatment
is empirical and consists of a combination of
topical and systemic drugs (such as corticosteroids,
immunosuppressants, analgesia) and local wound care.
In our series, corticosteroids were considered as the
first line treatment for this two disseminated PG. It has
demonstrated its efficiency for acute and rapidly
progressive forms. Furthermore, in the case n°1, we
administered intralesional corticosteroids. Both
patients were good responders and we did not add
immunosuppressant agents.
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