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Diagnosing Cutaneous Polyarteritis Nodosa After 17 Years Of Failed Wound Care

When a patient presents with several years of unsuccessfully treated wounds, the provider must consider a wider range of diagnoses. This author discusses an interesting case that shows how biopsy and multidisciplinary collaboration can make a significant difference. 

A 45-year-old female presented to my office with chronic ulcers of both legs present intermittently for approximately 17 years. During this time, she saw multiple physicians and was treated with multiple modalities, but had no long-term resolution. The ulcers were well-circumscribed with epibole at the wound margins. The bases of the ulcers were mostly granulation tissue with necrotic tissue, fibrotic tissue and biofilm scattered throughout. There was no surrounding erythema, proximal streaking, purulence, odor, crepitation or fluctuance. The ulcers were painful to palpation with minimal serous drainage.

The surrounding skin had dark scarring from previously healed ulcers and livedo reticularis. Both legs had small palpable nodules and mild non-pitting edema.  Pedal pulses were audible and triphasic with a handheld doppler. Minimal small varicosities were evident at the malleoli, bilaterally. Examination revealed decreased sensation to light touch in both lower extremities with mild foot drop, more so on the left than the right. The patient’s ambulation and station were unsteady with a steppage gait present. 

The patient’s past medical history included Graves’ disease, human immunodeficiency virus (HIV) and paranoid schizophrenia. She smoked and had a long history of polysubstance abuse. The patient had a body mass index (BMI) of 29.3, a temperature of 98.5 degrees Fahrenheit, pulse of 82 beats per minute, a respiratory rate of 18 breaths per minute and blood pressure was 142/82 mmHg.  

Wound cultures showed no growth. A venous doppler was negative for lower extremity deep vein thrombosis (DVT). Bilateral leg X-rays did not show any pathology and chest X-rays were also clear. A complete blood count (CBC) showed anemia and thrombocytopenia. The patient’s chemistry panel was within normal limits. The bloodwork also showed an elevated erythrocyte sedimentation rate (ESR) at 54 millimeters/hour and the C-reactive protein was elevated at 104.5 micrograms per milliliter. Hemoglobin A1c was normal at 5.5 percent. Ulcer tissue biopsy confirmed a diagnosis of polyarteritis nodosa.

What You Should Know About Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is an autoimmune disorder that causes necrotizing arteritis of medium or small arteries. This disorder does not cause glomerulonephritis or vasculitis in venules, arterioles, or capillaries. Polyarteritis nodosa is also not associated with antineutrophil cytoplasmic antibodies, which is otherwise a reliable marker for a pathologically distinct category of small vessel vasculitis.1

Polyarteritis nodosa can involve the skin, a single organ or may be present systemically. Although it can affect any organ, the disorder typically spares the pulmonary and glomerular arteries.1 Polyarteritis nodosa can be idiopathic or associated with viruses such as hepatitis B or HIV.

It is a very rare disorder with an estimated rate of three to 4.5 cases per 100,000 people in the United States. Polyarteritis nodosa is most likely to affect people between 45 and 65 years old, but it can occur at any age. This autoimmune disorder is also more common in men than women.2

The initial presentation of the disorder usually involves the skin or the peripheral nerves. Skin manifestations can include livedoid purpura, subcutaneous nodules and necrotic ulcers. Neurological signs include mononeuritis multiplex, such as wrist or foot drop. Other subacute symptoms include fever, weight loss, malaise, headaches or myalgia.3

Diagnosis can be difficult. There is currently no diagnostic laboratory test for polyarteritis nodosa. However, labs can help determine the extent of organs affected and their degree of involvement, including serum creatinine, muscle enzyme concentrations, liver function studies, hepatitis B and C viral serologies, urinalysis, ESR and C-reactive protein concentrations.3

If a patient meets three criteria established by the American College of Rheumatology, there is sensitivity and specificity of 82 and 87 percent for the diagnosis of polyarteritis nodosa. These criteria include:

• unexplained weight loss greater than four kilograms

• livedo reticularis

• testicular pain

• myalgias (excluding shoulder and hip)

• weakness of muscles

• tenderness of leg muscles

• mono- or polyneuropathy

• new onset diastolic blood pressure greater than 90 mmHg

• elevated blood urea nitrogen (BUN) or creatinine

• evidence of hepatitis B infection

• arteriographic abnormalities not resulting from non-inflammatory disease process and

• a biopsy of a small- or medium-sized artery containing polymorphonuclear cells.4

Systemic treatment of polyarteritis nodosa focuses on reducing inflammation and consists mainly of using corticosteroids, such as prednisone. Severe cases of the disorder may require the addition of chemotherapy medications (cyclophosphamide) or other immunosuppressants (methotrexate or azathioprine) that block abnormal growth of certain cells. When the polyarteritis nodosa is associated with hepatitis B, the treatment will include antiviral medications and possible plasma exchange.2 Five-year survival is 13 percent for those who are untreated versus 80 percent for those who are treated.3 Survival rates are lower for patients with hepatitis B virus-associated PAN.3

In regard to topical approaches to polyarteritis nodosa, there is no standard treatment. At this time, no prospective trials have been completed. Clinicians have to tailor therapeutic decisions to the severity of the skin lesions and their associated symptoms.5

Final Notes

Ulcers associated with polyarteritis nodosa are very rare and can be difficult to diagnose without a biopsy. Due to the systemic nature of the disease, consults with other specialties, including rheumatology, internal medicine and radiology, are essential. In addition, due to the risk of progression, strict follow-up is necessary. 

Dr. Swain is a board-certified wound specialist physician (CWSP) of the American Board of Wound Management, and a Diplomate of the American Board of Podiatric Medicine. He is the Medical Director of the St. Vincent’s Wound Care and Hyperbaric Center at St. Vincent’s Southside Hospital in Jacksonville, Fla., and is in private practice at the First Coast Cardiovascular Institute in Jacksonville, Fla.

Online Exclusives
By David Swain, DPM
References

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.

2. Polyarteritis Nodosa. Available at:  https://www.vasculitisfoundation.org/education/forms/polyarteritis-nodosa/#1545067261432-811a0766-7edd . Accessed January 15, 2020.

3.   Howard T, Ahmad K, Swanson JAA, Misra S.  Polyarteritis Nodosa. Tech Vasc Interv Radiol. 2014;17(4): 247–251.

4. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088–1093.

5.  Micheletti R. Cutaneous polyarteritis nodosa. Available at: https://www.uptodate.com/contents/cutaneous-polyarteritis-nodosa?search=polyarteritis%20nodosa&source=search_result&selectedTitle=3~150&usage_type=default&display_rank=3 . Accessed January 15, 2020. 

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