Treatment-resistant pyoderma gangrenosum
Published Web Locationhttps://doi.org/10.5070/D30k84c2vd
Letter: Treatment-resistant pyoderma gangrenosumCenter for Dermatology Research, Departments of Dermatology1, Pathology2 and Public Health Sciences3, Wake Forest University School of Medicine, Winston-Salem, North Carolina
Payman Kosari1 MD, Steven R Feldman1,2,3 MD PhD
Dermatology Online Journal 18 (4): 8
Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis that can have a chronic course often leading to ulceration and extreme tenderness. Treatment is often directed toward reducing the inflammatory process to prevent progression of the ulcer and minimize pain. The mainstay of therapy is systemic corticosteroids. Other immunosuppressants have been utilized in cases of PG resistant to corticosteroids, including cyclosporine and tacrolimus. A patient with severe and recalcitrant PG was prescribed systemic corticosteroids and cyclosporine but continued to have progression of her disease warranting admission to the hospital. Her hospital course was complicated requiring hospitalization for one month. Although her health was deteriorating, her PG improved in the hospital setting with corticosteroids without concomitant cyclosporine. The patient admitted to non-compliance with her medication in the outpatient setting, attributing her behavior mainly to depression. Pyoderma gangrenosum can be recalcitrant to any form of therapy and medication non-adherence must be considered a potential cause.
Systemic corticosteroids are a mainstay of treatment for PG. Adjunct or alternative therapy can be used in cases resistant to corticosteroids. Occasionally, medications fail in yielding results and other therapeutic options must be considered. Medication non-adherence can underlie treatment failure [1, 2, 6, 7]. Asking the patient may provide insight into their medication adherence behavior. We present a patient with therapy resistant PG secondary to medication non-adherence.
A 58-year-old woman was initially hospitalized in June 2011 for a non-healing ulcer on the right medial leg and presumed cellulitis. She was receiving vancomycin at the time that the dermatology service was consulted. A biopsy of the lesion showed an ulcer with exuberant granulation tissue and a neutrophilic infiltrate, consistent with PG. Further questioning and a complete work-up did not identify an etiology for the PG. Her antibiotic was discontinued and she was started on prednisone 60 mg, by mouth, daily. She was eventually stabilized and discharged to home with close follow up with dermatology.
She was seen in our department with worsening disease; despite continued prednisone plus the addition of cyclosporine 400 mg daily, she continued to worsen. Physical exam revealed a sad individual in extreme pain, crying during the whole encounter. She had a large 20 cm x 15 cm deep ulcer exposing the subcutis and underlying muscle. The ulcer extended from the right medial thigh to the right leg with exuberant granulation tissue. There was a surrounding erythematous to violaceous, indurated and undermined border. She had a similar but smaller 5 cm x 4 cm ulcer on the right lateral heel. The left leg was unaffected. The decision was made to admit her to the hospital for pain management.
On admission, she was started on methylprednisolone 80 mg intravenously, three times daily and cyclosporine 200 mg, by mouth, daily. She was receiving sodium hypochlorite solution dressing changes to her wounds three times daily. Her hospital course was complicated, requiring transfer to the medical intensive care unit (MICU) for hypotension secondary to Escherichia coli sepsis. Her corticosteroid was subsequently decreased to prednisone 100 mg, by mouth, daily and the cyclosporine was discontinued. Her health continued to decline and she suffered a myocardial infarction while in the MICU. With appropriate antibiotics and careful monitoring, she was transferred to the general medicine service only to return to the MICU secondary to a large pulmonary embolus involving the right and left main pulmonary arteries. Fortunately, she survived these episodes, was stabilized and transferred back to the general medicine service.
She continued to receive appropriate therapy for her PG during her admission. Her ulcers were improving as evidenced by a reduction in the erythematous, indurated borders and a considerable decrease in pain. When questioned in the hospital, the patient admitted to medication non-adherence before her admission. She was not taking her prednisone or cyclosporine four to five days of the week. Her reasons included depression regarding her disease, a lack of belief that the medicines would be effective, and confusion regarding how to take them. All issues were addressed before discharge from the hospital.
She has since followed-up in our dermatology clinic. She currently takes cyclosporine 300 mg daily and her prednisone was recently decreased to 50 mg daily. She states that she is compliant with our recommendations and continues to improve on this regimen.
Pyoderma gangrenosum is a sterile ulcerating neutrophilic dermatosis that can be extremely painful and treatment resistant. It can be disfiguring, negatively impacting quality of life (QOL) . One would incorrectly assume that these factors alone are enough to ensure medication adherence. In fact, there may be a negative correlation between QOL/disease severity and medication adherence . Our patient had severe progressive, painful and disfiguring PG but failed to adhere to our treatment regimen.
Adherence to medical treatment regimens for all diseases is poor and estimated to be the cause of 10 percent of hospital admissions . In dermatology, many factors contribute to non-adherence including forgetfulness, frequent dosing, and laziness, concern over side effects, and belief that the treatment will not be effective . Further complicating matters, patient adherence declines over time and patients may falsely, intentionally or unintentionally, over-report using their medications. Objective studies looking at medication adherence in psoriasis patients revealed that patients reported using their medications more than 90 percent of the time. However, according to data captured in electronic monitors, adherence decreased rapidly over the first few days and continued to decline at a rate of 20 percent every five weeks . Although initially reporting adherence, our patient was not taking her medications. Reasons included depression regarding her disease, a lack of belief that the medicines would be effective, and confusion regarding how to take her medication. She did not volunteer this information until late in her management. Since addressing these issues, her PG, and subsequently, her quality of life have improved.
Medication nonadherence should be considered in any skin condition unresponsive to appropriate therapy. If suspected, patients must be approached in a manner that makes them feel comfortable volunteering such information. Once, and if, it is established that non-adherence is contributing to disease recalcitrance, many actions can be taken to improve treatment outcome. Most importantly, the reason for that particular patients’ non-adherence must be determined and addressed. Other factors that may increase medication adherence include a discussion of side effects, clear instructions on how to take the medicine, and frequent follow-ups . It is important to keep in mind, however, that, even with an ideal approach, some patients will continue to exaggerate their adherence behavior making it extremely difficult to successfully treat them.
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