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Pyoderma Gangrenosum After Hand Surgery: A Case Report
Abstract
Introduction. Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis that can be triggered after minor trauma or surgery and mimics a fulminating infection. It is commonly associated with a systemic disease, such as inflammatory bowel disease, metabolic syndrome, rheumatologic or hematological disorders, and malignancies. The typical clinical appearance is hemorrhagic nodules, which rapidly progress into extremely painful, irregular, red to violaceous ulceration with undermined border and purulent necrotic bases. The treatment of PG is nonsurgical. Unnecessary surgical procedures may incite a pathergic response, worsening the disease dramatically and potentially resulting in a limb amputation. Case Report. A report of PG, originally misdiagnosed as an infection after a carpal tunnel release, is presented. Conclusions. This case emphasizes the importance of early recognition of PG to provide a timely diagnosis and avoid unnecessary surgeries, which can result in devastating consequences.
Introduction
Pyoderma gangrenosum (PG) is a reactive, noninfectious, neutrophilic dermatosis of unknown origin. Pyoderma gangrenosum is a rare disease with an incidence of approximately 0.63 per 100000 and a median age at presentation of 59 years.1 In up to 70% of cases, PG occurs in association with a systemic disease, most commonly inflammatory bowel disease, metabolic syndrome, rheumatologic or hematological disorders, or malignancies.2 The clinical presentation is characterized by hemorrhagic nodules that develop either spontaneously or after trauma and rapidly progress into extremely painful, irregular, red to violaceous ulceration with undermined border and purulent necrotic bases. The diagnosis is made on the basis of clinical findings and medical history as there are currently no definitive laboratory or histopathological criteria for PG.3 On average, postsurgical PG appears 7 days after surgery at the surgical incision site and is an important differential diagnosis in infections following surgery.4
Case Report
An 81-year-old female was referred to the authors’ department with the characteristic symptoms of carpal tunnel syndrome (CTS): numbness and nightly paresthesia in the thumb, index finger, and middle finger of the right hand as well as difficulties with fine motor activities like buttoning shirts. Phalen’s sign was positive whereas Tinel’s was negative. The sonographic measurements of the median nerve showed a delta of 7 and a wrist-to-forearm ratio of 2. The neurophysiologic study demonstrated severe sensorimotor CTS with absent sensory potentials and motor latency to abductor pollicis brevis (APB) of 6.8 ms; the threshold defining abnormal is greater than 1.2 to 1.8 ms.5
The patient’s medical history included arterial hypertonia, hyperlipidemia, hyperuricemia, multinodular goiter, urinary incontinence, post-hepatitis B infection status, and PG in the gluteal region after a bursectomy were present. Previous therapies included both the oral administration of cortisone (40 mg per day) and wearing a positioning splint at night for 2 weeks. The conservative treatment brought noticeable relief of the symptoms, but the numbness remained. After 14 days of treatment, it was decided to suspend the medication with cortisone and to proceed with an open carpal tunnel release (CTR) with wide-awake local anesthesia.
An experienced hand surgeon performed the CTR of the right hand without complication, and the patient was discharged to the outpatient treatment on postoperative day 1. A week after the surgery, the patient was referred to the authors’ outpatient clinic by the patient’s general practitioner with a wound presenting redness and swelling (Figure 1). Following the diagnosis of wound site infection, the patient was immediately admitted to the authors’ department. On the day of admission, the patient underwent a second surgery with debridement of the skin necrosis (Figure 2A). A proximal prolongation of the incision was performed, the Loge de Guyon released, and a drain inserted (Figure 2B). In spite of the appropriate wound debridement and antibiotic treatment, the lesion continued to deteriorate (Figure 3). Staining for microorganisms were negative and cultures were sterile. Hence, a dermatological consultation followed, and PG was diagnosed. The patient was consequently transferred to the Department of Dermatology, Venereology and Allergology at the Medical University of Innsbruck in Austria. The patient was treated with methylprednisolone (80 mg daily) in addition to antibiotic therapy, which led to a significant reduction of the swelling and red to violaceous coloration, cessation of lesion expansion, and eventually, wound reepithelization. The patient was discharged from the inpatient treatment 3 weeks after admission to the authors’ department. Treatment with glucocorticoid (20 mg cortisone) was gradually reduced (10 mg per day in alternation for 2 weeks, after which 10 mg for 2 weeks) and eventually stopped 7 weeks after the second surgery. The first follow-up was 2 weeks after hospital discharge. Further follow-ups occurred every 3 months. The follow-up examinations after discharge occurred for 7 months; there was an inconspicuous scar on the palm as well as an improvement of the sensitivity of the thumb and the fine motor skills (Figure 4).
Discussion
In 1983, the first case of PG on the hand was reported.6 It presented as an acute suppurative hand infection, which was unresponsive to drainage and antibiotics.6 Pyoderma gangrenosum may develop spontaneously or may be triggered by a minor trauma or surgery at any location of the body surface. Due to the clinical features of PG mimicking pyogenic wound infections, post-surgical PG is often initially misdiagnosed as an actual wound site infection.4 Hence, antibiotic therapy and unnecessary wound debridement not only fail to curb the extension of the ulcer, but they could also worsen its conditions by inciting a pathergic response.7 Pathergy phenomenon is a state of an enhanced inflammatory response of the skin after any cutaneous trauma.8 Incorrect therapy on the grounds of misdiagnosis may result in devastating consequences, including limb amputation.7,9 Lack of specific clinical, histopathological, and laboratory benchmarks makes PG a diagnostic and therapeutic challenge for surgeons. The diagnosis of PG in the present case was made after a consultation with a dermatologist and corticosteroid had been prescribed, with an appropriate clinical response. Successful systemic therapies have been reported with glucocorticoids, azathioprine, cyclosporine, tacrolimus, mycophenolate mofetil, methotrexate, chlorambucil, thalidomide, colchicine, cyclophosphamide, dapsone, minocycline, sulfapyridine, and tumor necrosis factor alpha inhibitors.10
Postsurgical PG is a rare but serious complication of surgery and is difficult to recognize. It is often initially diagnosed as a postoperative infection. In this particular case, the patient had a history of a trochanterica bursitis with a rupture of the gluteus medius muscle. After the surgical treatment by bursectomy and reattachment of the torn muscle back onto the greater trochanter, the patient had to undergo a revision and was transferred to the department of infectious diseases for the further antibiotic treatment. The present case report illustrates that despite the patient’s history of confirmed PG in the gluteal region after the bursectomy and reattachment of the gluteus medius muscle, the hand surgeons, who did not recognize the presentation of this condition, allowed the patient to undergo a CTR. Surgeons should consider PG in cases of rapidly enlarging ulcers that are unresponsive to antibiotics and aggravated by repeated debridement, so as to prevent a delayed diagnosis and to start an adequate therapy at an early stage. Especially in patients with prior episode of PG, this diagnosis should be high on the differential diagnosis if the surgical incision appears to be “infected.” For patients with PG in their medical history, dermatological consultation should be initiated prior to surgeries in order to carry out immunosuppression with steroids to avoid a recurrence.
Limitations
This is a single case report. Further clinical research is needed to understand the pathophysiology of PG, develop standardized diagnostic tools, and establish a treatment algorithm.
Conclusions
This case report describes the history of a patient with PG. The disease was originally misdiagnosed as an infection after a CTR. This fact emphasizes the importance of knowing the clinical presentation of PG in order to allow its early recognition and, by doing so, provide a timely diagnosis and avoid unnecessary surgeries, which can result in devastating consequences.
Acknowledgments
Authors: Turkhan Mehdiyev, MD; and Robert Zimmermann, MD
Affiliation: Department of Plastic, Reconstructive and Aesthetic Surgery, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, Austria
Correspondence: Turkhan Mehdiyev, MD, Department of Plastic, Reconstructive and Aesthetic Surgery, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, Austria; turkhan.mehdiyev@tirol-kliniken.at
Disclosure: The authors disclose no financial or other conflicts of interest.
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