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Case Report  |   October 2019
Pyoderma Gangrenosum–Like Lesion in an Older Adult
Author Notes
  • From Ohio University (Dr Gau) Heritage College of Osteopathic Medicine (Dr Swindell) in Athens. 
  • Financial Disclosures: None reported. 
  • Support: None reported. 
  •  *Address correspondence to William R. Swindell, DO, PhD, MS, The Jewish Hospital, 4777 E. Galbraith Road, Cincinnati, OH 45236; and Jen-Tzer Gau, MD, PhD, Heritage College of Osteopathic Medicine, 355 Grosvenor Hall, Athens, OH, 45701-2979. Email: ws277814@ohio.edu; and gau@ohio.edu
     
Article Information
Geriatric Medicine
Case Report   |   October 2019
Pyoderma Gangrenosum–Like Lesion in an Older Adult
The Journal of the American Osteopathic Association, October 2019, Vol. 119, 700-703. doi:https://doi.org/10.7556/jaoa.2019.117
The Journal of the American Osteopathic Association, October 2019, Vol. 119, 700-703. doi:https://doi.org/10.7556/jaoa.2019.117
Abstract

Pyoderma gangrenosum (PG) is a rare but serious neutrophilic dermatosis characterized by ulcerative lesions with violaceous borders. The disease mainly occurs in young or middle-aged adults. The authors present the case of a 79-year-old female nursing home patient who had an idiopathic PG-like ulcer on her lower extremity. Initial treatment with 3 courses of antibiotics and topical wound care for assumed cellulitis failed to improve the lesion, but the ulcer healed rapidly after a 1-week course of prednisone therapy (15 mg/d). This case provides insights into the challenges that arise in the diagnosis and management of PG in an older patient with extensive comorbidities. It also highlights the importance of considering PG as a diagnosis for older patients with a distinctive inflammatory ulcer that does not respond to antibiotics and topical wound care, since timely treatment with low-dose steroids can lead to quick healing by aborting the underlying autoinflammatory process.

Pyoderma gangrenosum (PG) is an autoinflammatory condition characterized by tender pustules developing into hemorrhagic ulcers with characteristic violaceous/bluish borders. The disease, originally named geometric phagedenism1 and renamed pyoderma gangrenosum,2 is not due to infection as suggested by its name (“pyo” is the Latin root for “pus” and “gangrene” describes eating away of tissue). A rare disease with an incidence of 3 to 10 cases per million population per year,3 PG may occur at all ages, although the peak incidence is between 20 and 50 years.4 The diagnosis is essentially clinical and based on lesion appearance, although biopsy findings may be useful to exclude other causes (eg, infection).5 Despite the lack of structured clinical trials because of the condition's rarity, existing guidelines advise treatment of the underlying disease, local wound care, and high-dose systemic steroids (eg, 60-120 mg of prednisone per day).5,6 
Pyoderma gangrenosum has seldom been described in elderly persons, and few case reports have documented the challenges that arise in the diagnosis and management of PG in this population.7-9 Older adults are a challenging treatment population because it is often necessary to manage comorbid conditions while avoiding polypharmacy.10 These challenges can be particularly critical for the management of PG, which is known to co-occur with other conditions, such as inflammatory bowel disease and rheumatoid arthritis.5 Current PG management guidelines have not differentiated between old and young patients,6 but steroid use in older adults requires caution because of adverse effects.11 It is not clear whether attenuated steroid doses can be as effective as high-dose therapy for older patients with PG, although physicians may be concerned that low-dose treatment would not induce full remission. 
We present the case of an elderly female nursing home patient with an idiopathic PG-like ulcer on her lower extremity that did not resolve with antibiotics nor topical wound care. 
Report of Case
On October 30, 2017, we visited a 79-year-old white woman (weight, 53 kg; height, 60 inches) living in a nursing home. The reason for the visit was concern for possible cellulitis on her right lower extremity. She had a history of cognitive impairment, anger outbursts, and confrontations with staff and residents. Musculoskeletal examination demonstrated mild scoliosis with kyphosis, edematous tissue texture of the extremities, and bilateral arthritic joint changes in the hands and knees. Deep vein thrombosis of the left lower extremity was identified in August 2017 and managed with rivaroxaban (15 mg/d) for 6 weeks, but it was discontinued because of bleeding risk. The patient also had frequent injuries to her extremities, leading to skin abrasions and bruises. Those lesions had healed spontaneously within 1 to 2 weeks without infection, although the patient was treated with a 1-week course of cephalexin for cellulitis in October 2017. 
The October 30th examination revealed 2+ diffuse edema with warmth, tenderness, and reddish-purple coloration on the right lower extremity. The patient was given a diagnosis of cellulitis and was treated with 100 mg of oral doxycycline twice per day for 7 days followed by 500 mg of oral levofloxacin daily for 5 days. On November 11, a lesion with exudate had developed on the anterior right lower extremity, prompting us to begin a third antibiotic treatment course with clindamycin (300 mg 3 times per day for 7 days). 
On November 26, the wound had not improved and was notable for PG-like features (Figure 1A). There was substantial swelling (2+) with tenderness. A radiograph demonstrated soft tissue swelling but no osteomyelitis. A swab culture demonstrated rare white blood cells, gram-negative bacilli, and a polymicrobic mixture of aerobes but no predominant organisms. Urinalysis demonstrated pyuria (26 high-power fields), and urine cultures grew Escherichia coli. 
Figure
Presentation and successful treatment of a pyoderma gangrenosum–like lesion in a 79-year-old patient with cognitive impairment and frequent falls. (A) Lesion on the right lower extremity at follow-up (November 26, 2017). (B) The same right lower extremity lesion 2 weeks after the start of 15 mg of prednisone per day (December 10, 2017). (C) Healed lesion 7 weeks after the start of prednisone treatment (January 18, 2018). (D) Persisting right lower extremity discoloration due to arterial and venous vascular disease (January 18, 2018).
Figure
Presentation and successful treatment of a pyoderma gangrenosum–like lesion in a 79-year-old patient with cognitive impairment and frequent falls. (A) Lesion on the right lower extremity at follow-up (November 26, 2017). (B) The same right lower extremity lesion 2 weeks after the start of 15 mg of prednisone per day (December 10, 2017). (C) Healed lesion 7 weeks after the start of prednisone treatment (January 18, 2018). (D) Persisting right lower extremity discoloration due to arterial and venous vascular disease (January 18, 2018).
At this point, the diagnosis of PG was considered, and prednisone was initiated (15 mg/d or 0.3 mg/kg/d for 7 days). It was further decided to treat the patient concomitantly with cephalexin (500 mg, 2 times per day for 7 days) because urine cultures had demonstrated bacteriuria, and the wound culture had grown gram-negative bacilli, indicating potentially complicated PG with a secondary infection. 
On December 8, after 7 days of prednisone therapy, the wound had improved considerably (Figure 1B). Nursing home staff had not reported any exacerbation of cognitive impairment during treatment. Nonfasting blood glucose levels remained stable. Lower extremity edema was significantly improved (trace/1+). Reexamination of the wound on January 18 demonstrated nearly complete resolution (Figure 1C). There remained substantial discoloration in the right lower extremity, consistent with the patient's history (Figure 1D). At present, the patient has not had a recurrence. 
Discussion
This case illustrates unique aspects of PG diagnosis and management in an older adult with cognitive and physical impairments. While PG may often have a more classic presentation in younger patients,12 the presentation in the current case was complicated by venous insufficiency, edema, and likely secondary bacterial infection. Because these conditions are common in older adults, PG may be more challenging to recognize or it may be misdiagnosed as primary cellulitis or venous stasis ulcer. Our case further showed that relatively low-dose prednisone therapy can be effective. Prednisone doses greater than 60 mg/d are often used as first-line PG treatment,6 but the current case illustrates that a lower dose can be sufficient to promote rapid resolution while avoiding adverse treatment effects. 
Pyoderma gangrenosum can be more challenging to manage in older adults with increased risk of comorbidity and mortality,12,13 although few case reports have described the unique concerns of older patients.7-9 A previous study evaluated 23 patients with PG between the ages of 30 and 89 years (mean age, 62.8 years).13 The study observed 5 deaths, and among these individuals, the average age at diagnosis was much older (age 78.8 years) compared with other patients.13 This increased mortality rate may be due to increased comorbidities commonly seen in the older adult population,12 as well as decreased capacity to tolerate systemic corticosteroids.11 
To improve PG outcomes in older patients, manual therapy could also be used to facilitate healing, particularly among patients with lower extremity lesions and venous insufficiency.14-16 Lymphatic congestion can entrap inflammatory mediators at the wound site and prevent matrix formation and remodeling.14 To decrease lymphatic congestion, pump techniques can be applied distal to the wound site,17 leading to improved systemic circulation and thoracic duct flow.18 Such adjunctive treatments may improve underlying edema or enhance steroid wound penetrance, possibly reducing the required dose and/or treatment duration.19 
We adopted a practical strategy with treatment response as a diagnostic indicator following empiric steroid treatment for presumptive PG. Clinical appearance of a rapidly developing tender lesion with an irregular, violaceous, and undermined border (Figure 1A) is a major criterion for PG diagnosis; additionally, our patient exhibited minor criteria such as favorable steroid response and pathergy.5 We did not obtain an excisional biopsy of the lesion because of its rapid resolution after steroid therapy. The pathology report, if obtained, could have been useful to exclude other diagnoses, although histologic findings are not pathognomonic for PG. Increased pathergy risk in older patients with PG12 also cautions against performing a biopsy and may favor empiric steroid treatment for some patients. 
Although widely understood to be a rare condition, the prevalence of PG may be higher in older patients with lower extremity symptoms. One large study of 31,619 patients with chronic leg ulcers, for example, estimated that twice as many cases could be attributed to PG (3.0%) than to infection (1.4%), among other common causes, such as venous and arterial insufficiency (79.7%).20 Heath care professionals should thus be aware of PG as a differential diagnosis for lower extremity lesions, particularly when those lesions do not improve with antibiotics and wound care. 
Conclusion
This case report emphasizes the importance of considering PG as an early possible diagnosis in older adult patients presenting with nonhealing skin ulcers refractory to antibiotic therapy and wound care. In such patients, short-term, low-dose prednisone (15 mg/d or 0.3 mg/kg/d for 7 days) can be effective for the management of PG. 
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Figure
Presentation and successful treatment of a pyoderma gangrenosum–like lesion in a 79-year-old patient with cognitive impairment and frequent falls. (A) Lesion on the right lower extremity at follow-up (November 26, 2017). (B) The same right lower extremity lesion 2 weeks after the start of 15 mg of prednisone per day (December 10, 2017). (C) Healed lesion 7 weeks after the start of prednisone treatment (January 18, 2018). (D) Persisting right lower extremity discoloration due to arterial and venous vascular disease (January 18, 2018).
Figure
Presentation and successful treatment of a pyoderma gangrenosum–like lesion in a 79-year-old patient with cognitive impairment and frequent falls. (A) Lesion on the right lower extremity at follow-up (November 26, 2017). (B) The same right lower extremity lesion 2 weeks after the start of 15 mg of prednisone per day (December 10, 2017). (C) Healed lesion 7 weeks after the start of prednisone treatment (January 18, 2018). (D) Persisting right lower extremity discoloration due to arterial and venous vascular disease (January 18, 2018).