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Suspicious ulcerations about the lower extremity
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An 83-year-old patient was referred to the limb preservation clinic for complaint of ulcerations to his left leg of four weeks duration.
According to the patient, the ulcerations initially appeared as areas of redness accompanied by pain. His primary care doctor subsequently placed him on amoxicillin/clavulanic acid. He denies history of previous ulcerations.
 Patris Toney |
His past medical history included diabetes, hypercholesterolemia, venous insufficiency, hypertension, peripheral neuropathy and Parkinson disease. He denied any surgeries. His medications included B12 supplements, simvastatin, zaroxolyn, lansoprazole, furosemide and potassium. His diabetes was non-medicated. He was afebrile and his vital signs were stable. Physical examination revealed palpable pedal pulses bilaterally with diminished sensation. Ulceration with granular base and mild periwound necrotic appearance was present to the posterior leg with a primarily necrotic appearing ulceration to the lateral left leg. Mild periwound erythema was noted (figures A and B), as was lower extremity edema. Also present were multiple diffuse, non-painful necrotic plaques with surrounding erythema (figure C).
During treatment, a painful ulceration with a deep bluish hued necrotic appearance developed to the lateral right heel. Shortly after, three areas of ulcerations with periwound erythema and coinciding pain developed to the right leg (figures D and E). The erosive ulcerations quickly increased in size with the erythema expanding and leading the way.
What would your diagnosis be?
A. Arterial ulcer
B. Venous ulcer
C. Pyoderma gangrenosum
D. Brown recluse spider bite
CASE DISCUSSION
The answer is C — the patient was diagnosed with pyoderma gangrenosum. The initial ulcerations to the left leg healed uneventfully with weekly collagen dressings. The right leg sites were treated with topical hydrocortisone 1% cream. The patient was also started on a medrol dose pak, followed by prednisone 5 mg once daily until healed. Response to therapy was noted by resolution of the inflammation as well as decrease in the spread of the ulcerations and reduction in pain. Topical treatment was changed to silver alginate powder dressing with progressive healing (figures F and G).
Pyoderma gangrenosum is highlighted by an inflammatory onset with suspicious dermatologic changes. Without warning these painful, pustular, nodular or papular lesions appear manifesting as violaceous or deep bluish pigmentary changes with surrounding erythema or solely erythematous. The overall initial appearance is necrotic or gangrenous. The necrotic, undermining “roof” of the lesions typically reveals a necrotic or hemorrhagic base that may eventually give rise to granular tissue.
Figures A and B: Left leg ulcerations at time of initial presentation to the clinic. A) Posterior leg ulceration with granular base and mild periwound necrotic appearance and erythema. B) Necrotic appearance of the lateral leg ulceration with resolving erythema.
Figure C: Multiple necrotic pre-ulcerative lesions with surrounding erythema about the left lower extremity (red arrows). Initial ulceration about the right inner ankle area (white arrow). Necrotic lateral left leg ulcer (yellow arrow). The shape varies between punctate or having jagged or scalloped edges. Depth ranges from superficial to full thickness with deeper penetration to muscle, tendon and bone occurring less frequently. The size can involve large portions of affected extremities and can occur quickly. Most notably seen on the legs, especially anteriorly, these ulcerations can also occur on the hands, genitalia, face, abdomen and neck.
This elusive derangement of the integument has been associated with multiple disease processes. Inflammatory bowel diseases are more commonly associated. Idiopathic cases have also been diagnosed.
Pyoderma gangrenosum is thought to be a diagnosis by exclusion. Dependent upon locale and appearance at time of initial presentation, differential diagnoses include venous ulcers, arterial ulcers, brown recluse spider bites, necrotizing fasciitis, gangrene, squamous cell carcinoma, basal cell carcinoma and necrobiosis lipoidica diabeticorum. Soft tissue infection should not be overlooked.
During the inflammatory phase, these ulcerations are mostly unresponsive to conventional wound treatment measures. First line treatments need to be directed at calming the inflammation. Topical hydrocortisone cream or other stronger steroid creams may be applied to the wound and/or periwound area. Intralesional corticosteroids may be used and oral corticosteroids are often concurrently prescribed or administered intravenously depending on the severity and situation.
Figures D and E: Initial inflammatory stage. D) Periwound inflammation of the more distal ulceration sites. The more proximal ulcer after the inflammation has been halted. E) Lateral right foot ulceration with periwound necrotic appearance and inflammation. Figure F: Right leg ulcerations. Resolution of inflammation and progressive healing .
Figure G: Healed right lateral foot ulceration.
All photos courtesy of Patris Toney
Flare-ups or regression with recurrences are not uncommon. In refractory or aggressive cases, effective systemic treatments include cyclosporine, minocycline, dapsone, tacrolimus, azathioprine, sulfasalazine and many others. Topical anesthetic creams have proven effective for temporary relief of pain and help facilitate dressing changes.
Premature surgical intervention is best avoided, especially at the onset due to increased tissue damage and spreading of the lesion.
Pyoderma gangrenosum remains poorly understood and is an easily missed diagnosis. For patients diagnosed with pyoderma gangrenosum, vigilance is important to facilitate quick treatment of new ulcerations.
Patris Toney, DPM, MPH, is a Fellow at the Center for Lower Extremity Ambulatory Research, and a Fellow at the National Center for Limb Preservation, Ill.