International Journal of Clinical Case Reports 2015, Vol.5, No. 50, 1-3
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lower limbs due to protein S deficit and treated with
acenocoumarol since 2 years, presented with an anal
pain and superficial ulceration of the right buttock
with temperature. She denied a history of hematologic,
gastrointestinal, or arthritis disease. Physical
examination showed 3x2 cm ulcer in the right gluteal
region and anal abscess. It started as a painful
papule which progressed to a pustule and then an ulcer
with a yellowish serous discharge and irregular
violaceous swollen edges. The rest of the physical
examination was normal. Laboratory data showed
neutrophil leukocytosis and high CRP rate. Other
laboratory tests including blood glucose level, liver
and renal function tests, serum electrophoresis, ANA,
ANCA and urinanalysis were reported as normal.
Tuberculin test was negative. Ulcer swab culture for
aerobic, anaerobic pathogens was positive for
E. Coli
.
Surgical flattering of the abscess was performed and
intravenous antibiotics (amoxcilline 3 g/day) were
administered. Skin lesions progressed to the lumbar
region and right flank, and other ulcers appeared in
the neck. Bacteriological swab remained this time
negative and skin biopsy concluded to epidermal
ulceration with an inflammatory infiltrate of
neutrophils and mononuclear cells. Based on the
clinical and histologic features, a diagnosis of familiar
PG was retained and the patient was started with oral
prednisone (55 mg/day). Complete reepithelialization
of the ulcers occurred in 8 weeks leaving behind
puckered scars.
Discussion:
Pyoderma gangrenosum is a rare, chronic ulcerative
skin disease. The overall incidence of PG is estimated at
6/million in the population (Langan et al., 2012). No
specific laboratory or histologic
tests confirm the
diagnosis. Pyoderma
gangrenosum is a diagnosis of
exclusion, after ruling
out other etiologies of
cutaneous ulceration
such as infectious, malignant,
vasculitic, and factitial, and favorable response to
steroid therapy. PG ulcers can occur anywhere in the
body. The earliest lesion is a pustule that typically
persists and develops into a large painful ulcer, with
erythematousto-violaceous undermined, rolled borders.
Four distinct clinical variants of pyoderma
gangrenosum have been described: ulcerative (classic),
pustular, bullous, and vegetative (Kikuchi et al., 2015).
Although pyoderma gangrenosum has been reported in
otherwise healthy people, 50% of the cases are associated
with underlying hematologic, gastrointestinal, and arthritic
disorders as it is illustrated in the case n°1 in which
the patient had a past history of inflammatory bowel
disease (Von den Driesch, 1997).
To our best knowledge, only four reports of about
familial predisposition in PG have been published
earlier in English literature (Miller and Dooley, 1973;
Al Rimawi et al., 1996; Khandpur et al., 2001;
Boussofara et al., 2013). This is the second report
of familial PG in Tunisia.
The first Tunisian report was about siblings, male and
female, born to nonconsanguineous parents who
developed at the age of 9 and 19 years old with a
subsequent diagnosis of CVID and atypical clinical
presentation showing oral involvement mimicking a
pyostomatitis vegetans.
In our cases, the patient was not siblings, both female
and was born to consanguinesous parents. In both
reports, the parents were uninvolved which suggests
autosomal recessive mode of inheritance.
Our observations were remarkable through the
spontaneous occurrence of the skin lesions. In fact, a
unique feature of PG is pathergy that is defined as an
inflammatory reaction in the skin induced by trauma,
and reportedly seen in 50% of PG patients (Satoh and
Yamamoto, 2013). No trigger factor was found in our
cases.
In the case n°2, the patient developed skin ulcers
while she was treated with oral anticoagulant (OAC)
for deep venous thrombosis. Other than the classic
skin necrosis induced by OAC in patients with
protein C and/or S deficiencies, recurrent pyoderma
gangrenosum (PG)-like ulcers induced by OAC was
previously reported and was the principal differential
diagnosis in this case. But, the chronology of the
events and the favorable outcome on steroids
treatment and the absence of recurrence while OAC
was not discontinued allowed a diagnosis of idiopathic
PG (Pralong et al., 2014).
The etiopathogeny of PG is still unclear. Its
association with a variety of immunologic disorders
and reports of family cases suggests an underlying
immunogentic defect. In fact, this was documented in
PAPA’s syndrome (Pyogenic sterile arthr itis,
