Pyoderma gangrenosum complicating bilateral breast reduction

Abstract

Pyoderma gangrenosum (PG) is an idiopathic disorder characterised by expanding areas of necrotic skin ulceration. PG's appearance is very similar to a necrotising bacterial infection; however, it will not respond to antibiotic therapy, and surgical debridement may cause progression of the disease. For this reason early diagnosis is important. We describe a case of PG presenting after bilateral breast reduction, in which bacteria were cultured, making diagnosis difficult. PG must be considered in poorly healing wounds even if bacteria have been isolated. A previously well 21-year-old Caucasian woman underwent bilateral, inferior pedicle breast reduction in November 2002. On the ninth post-operative day her sutures were removed and it was noted that she had areas of wound dehiscence, which were discharging pus. Swabs taken grew Staphylococcus aureus and coliforms and she was treated with oral flucloxacillin and metronidazole. She was readmitted 11 days later because of continuing wound problems. On admission she was apyrexial, but blood tests revealed a white blood cell count of 12.8×109/l (normal range 4.0–11.0) with a neutrophil count of 9.1×109/l (normal range 1.5–7.0). The wounds were irrigated. Swabs taken at this time grew Pseudomonas aeruginosa and she was treated with intravenous gentamicin. Over the following 3 months she had several wound debridements, vacuum pump therapy and underwent split skin grafting to the areas three times. The grafts seemed at first to heal, but eventually they all failed although the donor sites healed without problem. Further wound swabs grew MRSA, which was treated with a course of linezolid. The patient remained well apart from exquisitely painful wounds and an intermittent low-grade pyrexia. Because of the recurrent failure of the grafts after initially taking, pyoderma gangrenosum was then suspected, an opinion reinforced after dermatologic consultation. (Fig. 1—appearance at time of diagnosis). Wound biopsies revealed vasculitis and neutrophils in the ulcer edge compatible with the diagnosis of PG, so the patient was commenced on a trial of oral prednisilone 60 mg daily and topical betnovate cream twice daily. There was diminished pain and a noticeable improvement within 5 days, and the patient was discharged from hospital 5 days later on a reducing dose of prednisilone. A month later the patient started to develop side effects from the prednisilone and cyclosporin 150 mg twice daily was commenced, and the prednisilone tailed off rapidly. The wounds continue to heal well (Fig. 2) .Figure 2Healing wound following 6 weeks of immunosuppressant therapy.View Large Image Figure ViewerDownload (PPT) The cause of pyoderma gangrenosum has remained unclear since Brunsting et al. first described the disease in 1930,1.Brunsting L.A. Goekerman W.H. O'Leary P.A. Pyoderma gangrenosum: clinical and experimental observations in five cases occurring in adults.Arch Dermatol. 1930; 22: 655-680Google Scholar but it is likely that PG is due to dysregulation of the immune system. Pyoderma gangrenosum usually presents in association with systemic disease, most commonly ulcerative colitis, Crohns disease, rheumatoid arthritis and monoclonal gammopathies, but in up to 50% of cases there is no associated disease process at presentation.2.Von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up.Br J Dermatol. 1997; 137: 1000-1005Google Scholar The diagnosis of PG is one of exclusion. There are a range of presentations from an acute form of PG, which is usually associated with a systemic inflammatory response and can be rapidly developing, to a more chronic form, in which the patient is essentially well with slower ulcer development. In all cases, patients eventually develop extremely painful ulcerating lesions. Standard wound care and antibiotics are ineffective and debridement may actually worsen the ulceration.3.Rand R.P. Brown G.L. Bostwick III, J. Pyoderma gangrenosum and progressive cutaneous ulceration.Ann Plast Surg. 1988; 20: 280Google Scholar, 4.Brown R.E. Lay L. Graham D. Bilateral pyoderma gangrenosum of the hand: treatment with dapsone.J Hand Surg. 1993; 18B: 119Google Scholar The classic findings on examination are ulcers with pus-covered, necrotic centres (hence the term ‘pyoderma gangrenosum’), and ragged, undermined, violaceous edges. However, this clinical picture can vary. Other clues to the fact that this may be PG is sparing of the areolae5.Havlik R.J. Giles P.D. Havlik N.L. Plast Reconstr Surg. 1987; 80: 648Google Scholar (as in this case) and suture lines.6.Berry M.G. Tavakkolizadeh A. Sommerlad B.C. Necrotizing ulceration after breast reduction.J R Soc Med. 2003; 96: 186-187Google Scholar The histological picture of PG lesions is also variable, although there is usually a lymphocytic or neutrophilic vasculitis in the periphery of the wound. In most surgical contexts the diagnosis to exclude is infection. In the case described here several bacteria were cultured from the wound at different times confounding the diagnosis. None of the four previously reported cases of PG following BBR grew any organisms on culture of the wounds. Infections have been described in PG in other contexts, however, this has always been in patients with co-morbidity. In this case the patient was otherwise well, as is often the case in surgical patients, but as with other cases an important clue to diagnosis was severe and disproportionate pain from the lesions. The basis of management of PG is to control the disease process, usually with immunosupression. A prompt improvement of the wounds following a trial of immunosupressive treatment may confirm the diagnosis. The mainstay of treatment is systemic therapy, usually with high dose prednisilone, which can be supplemented by topical betnovate applications. There is also a report of successful treatment with intradermal injections of triamcinilone without the use of systemic immunosupression.7.Clugston P.A. Thompson R.P. Schlappner O.L.A. Pyoderma gangrenosum after reduction mammoplasty.Can J Surg. 1991; 34: 157-161Google Scholar Hyperbaric oxygen therapy has been used instead of immunosupressives with some success,8.Davis J.C. Landeen J.M. Levine R.A. Pyoderma gangrenosum: skin grafting after preparation with hyperbaric oxygen.Plast Reconstr Surg. 1987; 79: 200Google Scholar however, this is not easily available in most cases. The surgical management of PG wounds is controversial. Simple debridement and grafting of PG ulcers, as was done in this case initially, yields disappointing results and can even exacerbate the pathology. However, if conservative management is followed and these wounds are left to heal by secondary intention, this produces a characteristic and unsightly ‘parchment paper’ scar. Of the four other cases of PG occurring following BBR three of them were treated conservatively with residual scarring.6.Berry M.G. Tavakkolizadeh A. Sommerlad B.C. Necrotizing ulceration after breast reduction.J R Soc Med. 2003; 96: 186-187Google Scholar, 7.Clugston P.A. Thompson R.P. Schlappner O.L.A. Pyoderma gangrenosum after reduction mammoplasty.Can J Surg. 1991; 34: 157-161Google Scholar, 9.Gudi V.S. Julian C. Bowers P. Pyoderma gangrenosum complicating bilateral mammaplasty.Br J Plast Surg. 2001; 53: 440-441Google Scholar In one case the wounds were revised with peri-operative steroid cover giving a reportedly good result,3.Rand R.P. Brown G.L. Bostwick III, J. Pyoderma gangrenosum and progressive cutaneous ulceration.Ann Plast Surg. 1988; 20: 280Google Scholar however, the use of autografts in PG in other contexts have had mixed outcomes.5.Havlik R.J. Giles P.D. Havlik N.L. Plast Reconstr Surg. 1987; 80: 648Google Scholar If the aesthetic outcome is likely to be poor from conservative management and surgery is being considered there are three recommendations in the literature. Firstly, always use immunosuppressive cover and tail off the immunosupressants over a period of at least 6 months after surgery.10.Kaddoura I.L. Amm C. A rationale for adjuvant surgical intervention in pyoderma gangrenosum.Ann Plast Surg. 2001; 46: 23-28Google Scholar Secondly, if scar revision is being undertaken avoid placing epidermal sutures, use suture tapes instead.11.Long C.C. Jessop J. Young M. Holt P.J.A. Minimising the risk of post-operative pyoderma gangrenosum.Br J Dermatol. 1992; 127: 45-48Google Scholar Finally, if grafting is contemplated, use an allograft, with steroid cover, to minimise the pain and promote wound healing. If the allograft performs well then autografting can be considered.5.Havlik R.J. Giles P.D. Havlik N.L. Plast Reconstr Surg. 1987; 80: 648Google Scholar Although PG is likely to be encountered rarely by surgeons, it needs to be thought of in managing ‘difficult’ wounds, even if bacteria are cultured from the wound. PG can be indistinguishable in appearance to a necrotising infection; however, it will not respond to wound care and antibiotics, may cause disproportionate pain, and significantly, be worsened by debridement. Diagnosis of PG may only be confirmed by the success of a trial of immunosuppressive treatment.