The Importance of Early Diagnosis of Pyoderma Gangrenosum: A Case After Plastic Surgery

Pyoderma gangrenosum (PG) belongs to the deep or hypodermal group of neutrophilic dermatoses.¹ With a low incidence rate of 5 to 10 per 1 million, PG is a diagnosis of exclusion.² Its development after different types of plastic surgery (eg, carpal tunnel release, diagnostic breast biopsy, reconstructive breast surgery, and aesthetic breast surgery) has been described in the literature.^3-5 Owing to the significant overlap of symptoms with postoperative infection and skin necrosis, misdiagnosis is common. Patients are typically treated with antibiotics and surgical debridement. Nonetheless, surgical trauma leads to worsening lesions due to pathergy phenomenon.⁴ Herein is presented a bilateral case of PG localized to the breast after reduction mammoplasty with an excellent outcome owing to early diagnosis and intervention.

A 51-year-old female presented to the authors’ clinic with pain in the neck and back owing to excessive breast volume. On May 15, 2019, bilateral reduction mammoplasty with a superior medial pedicle technique was performed. The operation and early postoperative period passed without complications. Breast tissue in the amount of 1200 g and 960 g was excised from the right and left breast, respectively. The estimated blood loss was 50 mL.

Six days after the surgery the patient called the clinic, reporting drainage from the incisions, which became blood-tinged. She also reported that her breasts were red and warm to the touch. The authors examined the patient in the clinic on postoperative day 7. Bilateral breast erythema, swelling, minimal serpiginous drainage, and desquamation area at the base of the right areola with sloughing of epidermis were observed. Both nipple-areolar complexes were viable, however. Both breasts exhibited exquisite tenderness to palpation. The patient’s temperature was 100.9°F (38.3°C). The patient had no history of smoking or autoimmune disorder, and the family history was negative for autoimmune diseases. The medical history was significant only for the β-thalassemia trait, gastroesophageal reflux disease, sulfonamide allergy, and obesity. The patient had a body mass index of 36.18 kg/m². On postoperative day 7, the patient was admitted to the hospital with the diagnosis of possible cellulitis and was started on intravenous vancomycin and piperacillin-tazobactam. Petrolatum-based fine mesh gauze dressings measuring 4 cm × 4 cm were used to cover the wound. Despite the treatment, the wound did not improve, and the lesion progressed. 

Owing to the atypical location (ie, not confined to the incision site) and appearance (violaceous bulla at the 10-o’clock position, which later spontaneously burst) of the wound, a diagnosis of PG was considered (Figure 1). As a result, dermatology and infectious diseases specialists were consulted. The dermatology specialist recommended prednisone 1 mg/kg/day for management of PG, and ulcer biopsy was performed on May 24 (postoperative day 9). The culture results were negative. Nonetheless, pathologic analysis showed suppurative neutrophilic dermatosis consistent with PG, and infliximab (5 mg/kg in weeks 0, 2, 6, and 8) was started. Although systemic steroids and infliximab typically are used in patients with underlying inflammatory bowel disease, the patient in this case, who had no history of bowel disease, responded well to this treatment. After a 16-day hospital course, the patient was discharged on June 6 (postoperative day 22). The wound was partially healed by secondary intention with residual hypopigmentation by the 6th week (Figure 2). Complete healing with scarring was noted 2 months after initiation of treatment (Figure 3).

The exact etiology of PG remains unknown. Currently, the pathogenesis is believed to be multifactorial. The role of neutrophil dysfunction, inflammatory mediators, and genetic factors has been described in different studies. Each of these possible etiologies has been linked to specific PG subgroups. For example, PSTPIP1 gene mutations were found in patients with genetic syndrome–associated PG.²

Although PG lesions can occur in isolation, typically they are associated with underlying inflammatory conditions (in about 50% of patients) or autoimmune or malignant conditions (eg, inflammatory bowel disease, arthritis, hematologic malignancy, solid malignancy).^6-8 Alternatively, lesions may manifest as one aspect of a syndrome, such as pyogenic arthritis, PG, and acne (PAPA) or the PAPA-related syndromes pyogenic arthritis, PG, acne, and hidradenitis suppurativa ([PAPASH], originally known as PASS [PG, acne vulgaris, hidradenitis suppurativa and ankylosing spondylitis] and PG, acne, and hidradenitis suppurativa [PASH]).^6-8 

Among breast surgeries, reduction mammoplasty is the most frequent cause of postoperative PG, accounting for 44% of cases according to one systematic review.⁴ The presentation usually occurs between day 4 and week 4 after surgery.⁹ Late diagnosis of PG leads to additional surgical procedures, which cause additional damage, forming a vicious cycle resulting in increased hospital stay and costs. Severe consequences, such as disfigurement and partial loss of breasts, have been described.¹⁰ Skin grafting is required in some cases.¹¹ To prevent the aforementioned complications, different techniques have been suggested to diagnose PG earlier. For example, Neill et al¹² suggested using a simple mnemonic of the 5 P’s—painful, progressive, purple, pretibial, pathergy—to help clinicians with rare exposure to the pathology. 

In the present case, careful examination and determination of specific patient factors enabled diagnosis of PG at an early stage. Unlike in an infection, the wound had an ulcerative appearance. The lesion was not confined to the incision area; rather, it involved an oval area over the breast from the incision site to the nipples in an atypical distribution. In addition, the typical color changes of ischemic incision breakdown were not observed. The lesion had a purplish hue, undermined borders with no induration, and no granulation tissue. Moreover, culture results were negative. Finally, the patient’s condition worsened even though she was undergoing treatment with antibiotics.

Treatment options for PG can be divided into 3 main categories—topical, intralesional, and systemic. Class III and IV corticosteroids, calcineurin inhibitors, tacrolimus, clobetasol propionate, phenytoin, and tumor necrosis factor α inhibitor gel/infliximab are among the topical therapies described in the review by Quist and Kraas.¹¹ Triamcinolone acetonide and activated protein C can be injected intralesionally. Systemic treatment options include corticosteroids, cyclosporine A, mycophenolate mofetil, azathioprine, dapsone, chlorambucil, cyclophosphamide, thalidomide, methotrexate, clofazimine, and biologic agents. Despite the risk of deterioration due to surgical trauma, after controlling the active process, treatment such as negative pressure wound therapy and surgery such as skin grafting can be used to promote wound healing.¹¹ 

This study is limited because it reports only one case of PG. As a result, the present observations may not be generalizable. Additionally, the diagnostic indications described herein may not be applicable to all patients as the PG lesions may be atypical. Future studies are needed to assess the reproducibility of the present results. 

This case report highlights the importance of early diagnosis of PG. Awareness by plastic surgeons of the development of PG after breast surgery will help prevent detrimental complications, potential side effects of available treatment options, and patient suffering.

Authors: Gunel Guliyeva, MD¹; and Ali Kilic, MD, MSHA²

Affiliations: ¹Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, MD; ²UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL

Correspondence: Ali Kilic, MD, MSHA, UAB School of Medicine, University of Alabama at Birmingham, Surgery, JNWB 103, 1720 Second Ave South, Birmingham, AL 35294; akilic@uabmc.edu 

Disclosure: Patient data were accessed and extracted by Dr Kilic; Dr Guliyeva did not have access to the patient’s information. The authors disclose no financial or other conflicts of interest.