Eur J Plast Surg (2007) 30:41–43 DOI 10.1007/s00238-007-0128-8
CASE REPORT
Pyoderma gangrenosum: a difficult early diagnosis to make A. J. Lindford & D. G. Morritt & B. S. Dheansa & P. M. Gilbert
Received: 22 January 2007 / Accepted: 26 March 2007 / Published online: 24 April 2007 # Springer-Verlag 2007
Abstract Pyoderma gangrenosum (PG) is a rare, painful, non-infectious, ulcerative, inflammatory skin condition. It is characterised by ulcers that can spread rapidly showing undermined violaceous borders. It may develop at sites of trauma or in surgical wounds. Early diagnosis is not always easy as there is no single diagnostic test and clinical features are often indistinguishable from other more common ulcerative skin conditions. Diagnosis is made on clinical appearance, patient history and exclusion of other conditions. We describe a case of PG, which proved challenging to diagnose, in a man who presented initially with a non-healing leg ulcer at a site of previous trauma and surgery. He subsequently developed further lesions on the contralateral leg with the classical appearance of PG. PG is a condition, which may be encountered by plastic surgeons and should always be considered in the differential diagnosis of any ulcer. Keywords Pyoderma gangrenosum . Lower limb trauma . Fasciocutaneous flap
Presented at the Summer British Association of Plastic Surgery (BAPS) Meeting as a poster (July 13, 2006). A. J. Lindford : D. G. Morritt : B. S. Dheansa : P. M. Gilbert Department of Plastic and Reconstructive Surgery, Queen Victoria Hospital, East Grinstead, UK A. J. Lindford (*) 9 Lakeside Crescent, Brentwood, Essex CM14 4JB, UK e-mail:
[email protected]
Case report A 42-year-old man presented with an ulcer on his right leg. He had previous open right tibia and femur fractures sustained from a road traffic accident 20 years before this and had required fasciotomies and later coverage with a fasciocutaneous flap. His only other medical history consisted of mild asthma for which he used inhalers. He was also a non-smoker. He subsequently developed a small wound over the subcutaneous border of his right tibia in the region of the fracture. After 4 months, this wound had failed to heal and the patient was referred to the plastic surgery outpatient department. The wound was treated conservatively with dressings and healed completely. Eleven months later, the wound broke down leaving an ulcer measuring 2×0.5 cm. The ulcer was non-painful with undermined edges. Radiographs were taken and there was no evidence of osteomyelitis. An arterial Doppler ultrasound scan and MRI scan were performed, and both were normal. The ulcer was again treated conservatively with dressings. However, 2 months later, he presented to the Dressings Clinic with a 4 day history of painful new lesions on the contralateral leg. These were punched-out lesions with a purple edge and central slough (Fig. 1). These new lesions had the appearance of pyoderma gangrenosum (PG). Biopsies were taken from the ulcer’s edge and the patient started on prednisolone and co-amoxiclav. Blood tests were unremarkable with a white cell count of 7.5×109/l, normal urea and electrolytes and a C-reactive protein of 8 mg/l. An autoantibody screen was negative. Thyroid function tests did, however, suggest hypothyroidism with a raised thyroidstimulating hormone (TSH) and reduced T4 levels. He was subsequently started on thyroxine.
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Fig. 1 New lesion on contralateral calf and non-healing leg ulcer
The diagnosis of PG was later confirmed after review by a dermatologist. Lymecycline was then added, and the prednisolone dose was increased. Microbiological specimens taken from the ulcer revealed heavy growth of pseudomonas, scanty growth of Staphylococcus aureus and heavy growth of anaerobes. His antibiotics were subsequently changed to ciprofloxacin, flucloxacillin and metronidazole. A histological specimen taken from the edge of the ulcer showed no evidence of dysplasia or malignancy. The lesions were dressed regularly and healed completely within 4 months (Fig. 2).
Discussion In this patient, the diagnosis of PG was prompted by the development of new lesions on the opposite leg to the
Fig. 2 All wounds now fully healed
Eur J Plast Surg (2007) 30:41–43
original ulcer. All lesions healed after commencement of steroids. PG, first described by Brunsting et al. in 1930 [2], is an uncommon ulcerative cutaneous condition of uncertain aetiology, although immune system dysregulation may be involved. It mainly affects adults (men and women equally), predominantly between the ages of 25 and 54 years, affecting the lower limbs and trunk. It is characterised by painful ulcers that can spread rapidly with undermined violaceous borders and a necrotic, purulent base [10]. Clinical variants of PG include ulcerative, pustular, bullous and vegetative types. Histopathological findings in PG are non-specific but aids diagnosis by exclusion of other diseases. Microscopic features include massive neutrophilic infiltration, haemorrhage and necrosis of the overlying epidermis. These features may also be present in an abscess or cellulitis [3, 10]. In approximately 50% of cases, it is associated with an underlying systemic condition, most commonly, inflammatory bowel disease, rheumatic diseases, endocrine and haematological disorders [3]. However, PG may occur in healthy people and can develop after trauma or an operative procedure. This characteristic feature of PG is pathergy, a pathological hyper-reactivity to ‘normal’ stimuli [1]. The diagnosis of PG, based on clinical presentation, course and histology, is one of exclusion. Conditions that may mimic PG are: systemic vasculitis, rheumatic diseases such as Behcet’s syndrome, vascular (venous, arterial or neuropathic) ulcers, infections, malignancies or endocrine diseases such as necrobiosis lipoidica. If a biopsy is performed, it must be done as atraumatically as possible because it can provoke a pathergic response and worsen the condition [3, 10]. The treatment of PG is systemic therapy with prednisolone. It is important to control the disease quickly; so substantial dosages (40 to 120 mg daily, approximately 1 mg/kg) at the initiation of therapy are required. High-dose systemic steroids should be continued until each lesion is healed, and sometimes prolonged low-dose maintenance therapy is required to prevent recurrence. In steroidresistant cases, immunosuppressive drugs have been shown to be effective [3]. Tetracyclines have also been reported as being successful in the treatment of PG. A tetracycline such as minocycline or lymecycline is thought to act through its anti-inflammatory/anti-chemotactic properties [10]. Local management of the ulcers such as the application of anti-bacterial creams or topical sulfone and gentle daily lavage with saline have been reported. Hyperbaric oxygen therapy has also been reported to create an enhanced environment for wound healing [3, 10]. Owing to the phenomenon of pathergy, surgical treatment can worsen the condition. Hence, correct recognition and diagnosis of PG is of paramount importance [1, 3].
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Other cases of PG that have been encountered by plastic surgeons include a case occurring in a lower limb fasciocutaneous flap 1 month post-operatively [6]; a recurrence of PG in a foot free flap re-construction 5 weeks post-operatively [7]; 2 cases complicating bilateral breast reduction [4, 5] and another, which occurred after breast re-construction with a latissimus dorsi flap [9]. It is interesting to note that there were no documented cases associated with lower limb fractures, although this is of course a form of trauma. Long et al. [8] suggested that to minimise the risk of developing post-operative PG in patients with a history of the condition, surgery should be performed when the disease is quiescent and by using sub-cuticular sutures for skin closure to avoid puncturing the skin surface.
Summary PG is a rare condition, which may be encountered by plastic surgeons. This report should raise awareness of this sometimes difficult to diagnose yet readily treatable condition. PG should always be considered in the differential diagnosis of an ulcer.
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