Photo Quiz

Skin Ulcers of Unknown Etiology

 

Am Fam Physician. 2016 Jan 1;93(1):57-58.

A 41-year-old woman presented to the emergency department with leg ulcers and ecchymoses on her ears that began to develop one month prior. The leg ulcers began as painful, fluid-filled blisters and evolved into ulcers with a black crust. The lesions had appeared and resolved several times over the previous three years, but she did not seek medical attention. She took prednisone intermittently for psoriasis and had a long history of cocaine abuse.

Physical examination showed multiple ulcerated lesions on the anterior aspect of both lower extremities (Figure 1) and multiple tender purpuric lesions on both ears (Figure 2). The examination showed psoriatic plaques on her legs, elbows, and fingers. Her vital signs were normal. A complete blood count, comprehensive metabolic panel, and coagulation laboratory test results were normal. Urine toxicology testing was positive for cocaine. She had an elevated C-reactive protein level (96.3 mg per L [917.16 nmol per L]) and erythrocyte sedimentation rate (48 mm per hour).

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Figure 1.


Figure 1.

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Figure 2.


Figure 2.

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Levamisole-induced vasculitis.

B. Necrobiosis lipoidica diabeticorum.

C. Pyoderma gangrenosum.

D. Septic emboli.

E. Wegener granulomatosis.

Discussion

The answer is A: levamisole-induced vasculitis. A skin biopsy showed a leukocytoclastic vasculitis consistent with levamisole-induced vasculitis. Levamisole is an antihelminth drug that was used as an antineoplastic agent, but adverse effects such as agranulocytosis and an ulcer-causing vasculopathy have now limited its use to veterinary medicine. It is commonly used to lace cocaine because of its psychoactive effects. It is estimated that 70% of cocaine in the United States contains levamisole.1,2

The lesions associated with levamisole exposure are most commonly purpura on the ears, nose, and cheeks. Skin ulcers with a hemorrhagic base may also occur. These lesions usually resolve spontaneously within a few weeks of discontinuing the drug, but can recur with subsequent exposure.24

Necrobiosis lipoidica diabeticorum occurs in patients with diabetes mellitus or a strong family history of the disease. It is characterized by single or multiple asymptomatic red to yellow, shiny plaques that gradually enlarge and contain dermal blood vessels. Ulceration of the plaques is common and can occur with or without trauma. The pathogenesis is unknown, but biopsy can confirm the diagnosis.5

Pyoderma gangrenosum is an idiopathic condition associated with inflammatory bowel disease, arthritis, joint inflammation, and malignancy. It is characterized by painful pustules and rapidly progressive ulcers with violaceous undermined borders, surrounding erythema, and a purulent base. The pathogenesis is believed to be related to neutrophil dysfunction. It is a diagnosis of exclusion.5

Bacteria and pus from vegetations on an infected heart valve may cause septic emboli. They travel via the bloodstream and can cause the sudden development of painful, purpuric skin ulcers. They are associated with other systemic signs of bacteremia, including fever, malaise, myalgias, arthralgia, and elevated white blood cell count.6,7

Wegener granulomatosis is a small to medium vessel autoimmune vasculitis that is characterized by skin, lung, and kidney involvement. Skin findings include necrotic ulcers, palpable purpura, and digital infarcts. Patients with this condition have lung nodules, upper respiratory tract disease, and segmental necrotizing glomerulonephritis.5,7

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Summary Table

ConditionCauseCharacteristics

Levamisole-induced vasculitis

Levamisole exposure

Purpura on the ears, nose, cheeks; skin ulcers with a hemorrhagic base

Necrobiosis lipoidica diabeticorum

Unknown but associated with diabetes mellitus

Single or multiple asymptomatic red to yellow, shiny plaques; gradual enlargement and possible ulceration

Pyoderma gangrenosum

Neutrophil dysfunction

Painful pustules and rapidly progressive ulcers with violaceous undermined borders, surrounding erythema, and a purulent base

Septic emboli

Heart valve infection

Sudden development of painful, purpuric skin ulcers; other systemic signs of bacteremia

Wegener granulomatosis

Autoimmune vasculitis

Necrotic ulcers, palpable purpura, digital infarcts; skin, lung, and kidney involvement

Summary Table

ConditionCauseCharacteristics

Levamisole-induced vasculitis

Levamisole exposure

Purpura on the ears, nose, cheeks; skin ulcers with a hemorrhagic base

Necrobiosis lipoidica diabeticorum

Unknown but associated with diabetes mellitus

Single or multiple asymptomatic red to yellow, shiny plaques; gradual enlargement and possible ulceration

Pyoderma gangrenosum

Neutrophil dysfunction

Painful pustules and rapidly progressive ulcers with violaceous undermined borders, surrounding erythema, and a purulent base

Septic emboli

Heart valve infection

Sudden development of painful, purpuric skin ulcers; other systemic signs of bacteremia

Wegener granulomatosis

Autoimmune vasculitis

Necrotic ulcers, palpable purpura, digital infarcts; skin, lung, and kidney involvement

Address correspondence to Sommer Aldulaimi, MD, at sommer.aldulaimi@bannerhealth.com. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Metwally O, Hamidi M, Townsend L, Abualula H, Zaitoun A, Lall T. The cocaine trail: levamisole-induced leukocytoclastic vasculitis in a cocaine user. Subst Abus. 2013;34(1):75–77....

2. Pillow MT, Hughes A. Levamisole-adulturated cocaine induced vasculitis with skin ulcerations. West J Emerg Med. 2013;14(2):149–150.

3. Abdul-Karmin R, Ryan C, Rangel C, Emmett M. Levamisole-induced vasculitis. Proc (Bayl Univ Med Cent). 2013;26(2):163–165.

4. Lee G, Negash D, Arseneau R. Chronic levamisole-induced vasculitis: a case report. BCMJ. 2012;54(6):302–304.

5. Panuncialman J, Falanga V. Unusual causes of cutaneous ulceration. Surg Clin North Am. 2010;90(6):1161–1180.

6. Zembowicz A, Navarro P, Walters S, Lyle SR, Moschella SL, Miller D. Subcutaneous thrombotic vasculopathy syndrome: an ominous condition reminiscent of calciphylaxis: calciphylaxis sine calcifications? Am J Dermatopathol. 2011;33(8):796–802.

7. Suresh E. Diagnostic approach to patients with suspected vasculitis. Postgrad Med J. 2006;82(970):483–488.

This series is coordinated by John E. Delzell, Jr., MD, MSPH, Assistant Medical Editor.

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