International Journal of Clinical Case Reports 2015, Vol.5, No. 50, 1-3
1
Case Report Open Access
Familial Pyoderma Gangrenosum: About 2 cases
Nabil Bel Fek
Monia Smiti-Khanfir, Fatma Said, Imed Ben Ghorbel, Thouraya Ben Salem, Mounir Lamloum,
Amira Hamzaoui, Mohamed Habib Houman
Department of Internal Medicine, University Hospital of La Rabta, Tunis, Tunisia
Corresponding author email
International Journal of Clinical Case Reports, 2015, Vol.5, No.50 doi: 10.5376/ijccr.2015.05.0050
Received: 21 Jul., 2015
Accepted: 22 Aug., 2015
Published: 02 Sep., 2015
Copyright
©
2015 Feki et al., This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use,
distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Nabil Bel Feki, Monia Smiti-Khanfir, Fatma Said, Imed Ben Ghorbel, Thouraya Ben Salem, Mounir Lamloum, Amira Hamzaoui, and Mohamed Habib
Houman, 2015, Familial Pyoderma Gangrenosum: About 2 cases, International Journal of Clinical Case Reports, 5(50): 1
-
3
Abstract
Pyoderma gangrenosum occurrence in a familial pattern is extremely rare. We report pyoderma gangrenosum in two
Tunisian siblings with onset respectively at 28 and 26 years old. The initial lesion was a pustule that breaks down to form an ulcer
with an erythematous border. Treatment with oral corticosteroids induced an excellent clinical response. This familial clustering
suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.
Keywords
Pyoderma gangrenosum; Heredity; corticosteroids
Introduction
Pyoderma gangrenosum (PG) is an inflammatory,
ulcerative skin condition that occurs in all age groups.
Its occurrence in a familial pattern is extremely rare.
As far as we could ascertain, only four reports
indicating genetic predisposition have been published
earlier (1-4). We describe familial pyoderma gangrenosum
in two Tunisian sisters with unaffected parents
Clinical presentation
Case 1
A 28-year-old female patient, born to non consanguineous
parents, with past medical history of tuberculosis and
crohn’s disease complicated with parotid enlargement
and interstitial lung disease, presented with skin ulcers,
high temperature, and swollen joints. She reported that
during 2 week period the skin lesions started as
erythematous pustule in her back. Physical
examination showed multiple ulcers with undermined
violaceous margins and indurated bases on posteriorly
faces of both thighs and in the lumbar region. The oral
mucosa was unaffected. The rest of the clinical
examination was unremarkable. Biological investigations
showed biological inflammatory syndrome. Cell blood
count, serum protein electrophoresis, liver and renal tests,
glucose and electrolytes were normal. Immunological
investigations including antinuclear antibodies (ANA),
antiphospholipids antibodies, anti-neutrophil cytoplasmic
antibodies (ANCA) were negative. Blood culture, skin
swabs, urine analysis, BK sputum examination, and HIV
serology were all negative. The patient was initially
treated with intravenous antibiotics (pristinamycine 3
g/day, metronidazole 1.5 g/day, and gentamicin
160 mg/day) associated to surgical resection of the
necrotic tissue. Despite this first line treatment, skin
lesions rapidly progressed and the patient was pyrexic.
Histopathologic examination of the excised tissue
showed extensive ulceration and necrosis of epidermis
and dermis down to subcutaneous fat. A mixed cell
infiltrate extended from the papillary and reticular
dermis down the subcutis, and was composed of
neutrophils, lymphocytes, and prominent reactive
histiocytes. There was no evidence of vasculitis,
infection, or granuloma formation. Immunological
stain ruled out lymphoproliferative syndrome. The
histopathologic findings and the clinical picture were
considered consistent with pyoderma gangrenosum.
Treatment was commenced with oral prednisone (60
mg/day) and daily dressings with topical fusidic acid
and bethamehasone valerate. The area of ulceration
ceased to enlarge and became less inflamed. The
patient was afebrile but required transient morphine
analgesia. Oral prednisone was tapered slowly after 6
weeks of full doses. After 12 months of treatment, the
lesions have completely healed and the patient was
well and thriving.
Case 2
A 26-year old female patient, born to non consanguineous
parents, with past history of deep venous thrombosis of
