Inflammatory ulcers: Pyoderma gangrenosum

Recommendations

Identify and Treat the Cause
1 Diagnose and treat suspected pyoderma gangrenosum rapidly to prevent severe scarring. Level of Evidence
Not Assessed
2 Confirm the diagnosis and rule out other causes by clinical examination, a search for underlying conditions, culture and biopsy. Level of Evidence
Not Assessed
3 Implement combination therapy with a corticosteroid plus cyclosporine in patients requiring systemic therapy. Level of Evidence
Not Assessed
4 Implement aggressive treatment of any underlying condition. Level of Evidence
Not Assessed
5 Avoid wound trauma due to the potential for pathergy. Level of Evidence
Not Assessed
6 For nonhealable or maintenance wounds, provide support, pain control and modified local care (bacterial and moisture reduction) Level of Evidence
Not Assessed


Address patient-centered Concerns
7 Communicate (patients, family, caregivers) to establish a social support system with realistic expectations for healing and to prevent leg ulcer recurrences. Level of Evidence
Not Assessed
8 Assess / Control pain and optimize activities of daily living. Level of Evidence
Not Assessed


Provide Local Wound Care
9 Assess and document the wound at regular intervals. Level of Evidence
Not Assessed
10 Optimize local wound care: inflammation / infection control, and moisture balance. Consider biopsy, if appropriate, active (including biologicals) & adjunctive therapies if the wound is not healing at the expected rate. Level of Evidence
Not Assessed


Provide Organizational Support
11 Consult appropriate disciplines to maximize healing (e.g. mobility and nutrition). Level of Evidence
Not Assessed


Background

Pyoderma gangrenosum (PG) is a primary ulcerating inflammatory skin disease that has a sudden onset and may progress rapidly. A variety of immunologic abnormalities have been identified in people with PG, and these may play a role in pathogenesis. Approximately half of cases are associated with underlying systemic disease, especially inflammatory bowel disease, arthritis and myeloproliferative disorders. Four main types are described: classic, pustular, bullous and vegetative.
♦ Classic PG typically presents on the leg as a deep ulcer with a well defined, blue or purple undermined border. It may begin as one or more papules, which break down to form a cluster of small ulcers that coalesce into a single lesion. Nonspecific systemic symptoms, including fever, malaise, myalgia and arthralgia, may accompany the ulcer. Lesions are usually painful and sometimes severely painful. In up to half of cases, the lesion develops at the site of minor trauma. Ulcers are initially sterile but staphylococci and streptococci may be detected later.
♦ Pustular PG, which occurs in patients with inflammatory bowel disease and does not progress beyond the pustular stage, tends to occur on the trunk or extensor surfaces of the limbs.
♦ Bullous PG, which occurs primarily in patients with hematologic malignancies, is seen on the arms or face more frequently than on the legs. It usually presents as concentric bullae that spread rapidly and may ulcerate, generally forming superficial ulcers with an undermined blue border.
♦ Vegetative PG, a superficial form of disease, appears to be less aggressive than other types. It usually occurs as a single lesion in patients who are otherwise well and may respond to topical treatment.

Rapid diagnosis is important to prevent treatment delays and minimize scarring. Diagnosis is usually based on clinical features, as histology is nonspecific. Biopsy can exclude other conditions. Culture is recommended to rule out infection. Identification of any underlying systemic disease is critical, as effective treatment of the underlying condition may also improve the PD. Conditions that may resemble PG include vasculitis, malignancy, infection, necrotizing ulcers from spider bites, and pustular drug reactions.

Treatment is directed to halting ulcer progression, reducing pain, promoting healing and minimizing scarring. Treatment is empiric, as no guidelines currently exist. A systematic review indicated that the treatment of choice is a corticosteroid plus cyclosporine. Other immunosuppressive agents and dapsone have been used with mixed results. Infliximab has demonstrated benefit compared with placebo. Long-term immunosuppression is often necessary. The best sign of treatment efficacy is a decrease in pain.

When performing local wound care, it is important to minimize trauma, due to the potential for pathergy. As a result, surgical intervention and debridement should be avoided. High-potency topical corticosteroids and tacrolimus may also be useful, especially in conjunction with systemic therapy.

References

Essential Publications
1 Pyoderma Gangrenosum Quality Indicator
 Type: Narrative Review
Ehling A, Karrer S, Klebl F, Schaffler A, Muller-Ladner U. Therapeutic management of pyoderma gangrenosum. Arthritis and Rheumatism 2004: 50(10) 3076-3084.
Although few controlled trials of interventions have been performed on this topic, the information that has been collected in this review provides treatment options for clinicians to consider, including topical therapy, surgery, adjuvant and complement therapy, established systemic therapy and novel therapies. These therapeutic approaches can later be adapted to the specific problems of the individual patient.
2 Pyoderma Gangrenosum Quality Indicator
 Type: Narrative Review
Patel P and Topilow A (2002). Images in clinical medicine: Atypical pyoderma gangrenosum in a splenectomy. The New England Journal of Medicine 347 (18): 1419-1420.
Atypical pyoderma gangrenosum is a rare, idiopathic inflammatory disease that is easily confused with classic pyoderma gangrenosum. The dermal lesions of atypical pyoderma gangrenosum are more superficial than those of classic pyoderma gangrenosum and are frequently located on the arms rather than on the legs.
3 Pyoderma Gangrenosum Quality Indicator
 Type: Systematic review
Reichrath J, Bens G, Bonowitz A, Tilgen W (2005). Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol 53: 273-83.
This systematic review was undertaken to determine the extent and levels of evidence supporting treatment recommendations for Pyoderma Gangrenosum (PG). Therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented. The mechanism of action of thalidomide in Pyoderma Gangrenosum (PG) is unclear. Anti-inflammatory effects include inhibition of both macrophage phagocytosis and neutrophil chemotaxis. Topical treatment of PG should be primarily directed to avoid secondary wound infection. Antiseptic or occlusive wound dressings are the topical treatment of choice in these patients. Overall, more rigorous comparative trials are urgently needed for definitive conclusions with respect to PG treatment.
4 Pyoderma Gangrenosum Quality Indicator
 Type: Systematic review
Weenig RH, Davis MDP, Dahl PR, Su WPD (2002). Skin ulcers misdiagnosed as pyoderma gangrenosum. The New England Journal of Medicine 347(18): 1412-8.
The charts of 240 patients were reviewed. The objective of this study was to determine the frequency of misdiagnosis of pyoderma gangrenosum and to identify the causes of cutaneous ulceration and the usefulness of biopsy in patients who receive such misdiagnoses. The results show that the misdiagnosis of pyoderma gangrenosum is not uncommon (as high as 10%) and it exposes patients to substantial risks associated with its treatment, since cutaneous ulceration is a common manifestation of several vasculitides. It was found that Wegener’s granulmatosis was the most frequent cause of pyoderma gangrenosum-like ulceration. A thorough workup (including biopsy) is required in all patients suspected of having pyoderma gangrenosum in order to rule out diagnoses that mimic pyoderma gangrenosum.


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