Pyoderma Gangrenosum of the Breast After Mastopexy

Introduction. Pyoderma gangrenosum (PG) is a rare, inflammatory, noninfectious skin disorder that is idiopathic in nature; however, it may occur as a rare complication of breast surgery. The mainstay of treatment is medical immunosuppression. Case Report. This report describes the case of a 46-year-old woman who developed PG of both breasts following bilateral reduction mammoplasty and mastopexy. She was managed with a combination of medical immunotherapy and full-thickness skin grafts, which resulted in successful wound healing. Conclusions. This patient’s positive outcome illustrates the use of skin grafts in combination with medical immunotherapy in the setting of PG. This approach, which differs from the common method of treating solely with medical immunotherapy, may provide a quicker and more satisfying result for the patient.

Pyoderma gangrenosum (PG) is a rare, inflammatory, noninfectious skin disorder classified as a neutrophilic dermatosis.¹ The incidence of PG has been estimated to be 3 to 10 cases per million people every year.² The etiology of the disease is unknown, but it appears to be associated with systemic diseases such as inflammatory bowel disease.³ It also may arise rarely following surgery.  

The disease initially presents as an inflammatory nodule or pustule that progresses to an ulcerative lesion. The ulcer tends to have an irregular, elevated, and violaceous border with a necrotic and purulent base. On a histological basis, the prominent cell type in PG is the neutrophil. In addition to neutrophilic infiltration, features include edema, small- and medium-sized vessel engorgement and thrombosis, hemorrhage, and necrosis.¹ The pathophysiology of PG is unclear. However, it has been suggested that neutrophil dysfunction is involved in the process, specifically in the impairment of phagocytosis by neutrophils. Furthermore, overexpression of interleukin 8, which is a chemotactic agent targeted at neutrophils, has been found in PG ulcers. Overall, the neutrophil is involved in the immunological abnormalities present in PG, and the etiology of the disease is likely multifactorial with genetic, immunological, and inflammatory factors.⁴

Medical immunosuppression is the common management of PG. For limited or localized disease, local therapy including topical steroids and tacrolimus ointment are used. For more widespread and extensive disease, systemic corticosteroids are the first-line therapy. If these fail to improve the symptoms of the disease, immunosuppressive drugs, such as cyclosporine, or immunomodulatory drugs, such as tumor necrosis factor alpha inhibitors, can be added.⁴

Once a diagnosis of PG is made, there is caution in performing any type of surgical treatment due to the risk of pathergy that may accompany the disease.⁵ This leads to wounds being allowed to heal by secondary intention, which can take a significant amount of time. The use of skin grafts or flaps may provide a quicker approach to the healing of wounds affected by PG. 

Presented herein is the case of a patient who developed PG of the breast following mastopexy and reduction mammoplasty and was treated with a combination of medical immunosuppression, including prednisone and cyclosporine, and full-thickness skin grafts (FTSG). Ethics approval was obtained from the Dalhousie University Research Ethics Board (Halifax, Nova Scotia, Canada) and written informed consent for this report was obtained from the patient.

A previously healthy 46-year-old woman underwent cosmetic bilateral reduction mammoplasty and mastopexy at a private center in Halifax. She initially did well postoperatively but developed a fever of 39°C as well as excruciating pain and swelling in both breasts on postoperative day (POD) 3. On POD 5, she spoke to a general practitioner who diagnosed her with a postoperative infection and started her on oral clindamycin. Despite this, on POD 7, she continued to have increasing pain and swelling as well as drainage from both breasts. At this point, she presented to the emergency department in Halifax, where the emergency physician described her breasts as red, firm, and tender with areas of necrosis inferiorly (Figure 1). Blood tests revealed a white blood cell count (WBC) of 24.7 x 10⁹/L. Her vitals were measured at this time with a heart rate of 144 beats per minute, temperature of 36.9°C, and blood pressure of 112/63 mm Hg. She was thought to have a necrotizing skin infection and was started on intravenous ceftriaxone (1 g/day), clindamycin (900 mg, 3x/day), and vancomycin (1 g, 2x/day). At this point, she was referred to the plastic surgery department on an urgent basis. 

Once examined by plastic surgery, she was taken to the operating room (OR) urgently on POD 7 for debridement of the inferior necrotic skin flaps. Swabs were taken during the operation and sent for analysis. She was hospitalized postoperatively and initially did well. Various dressings were applied daily to the open areas of her inferior breast. The swabs from her operation showed growth of coagulase-negative Staphylococcus bacteria. The infectious diseases department was consulted, and it was felt that, given the state of the apparent infection, this was not the virulent pathogen; rather, it was likely to be a skin contaminant. 

She became febrile (39.1°C) on POD 10 and experienced a rise in her WBC over the following few days (19.6 x 10⁹/L on POD 10, 33.9 x 10⁹/L on POD 12, and 48.0 x 10⁹/L on POD 13). There was increased redness, ulceration, and necrosis in both breasts. She was taken to the OR again for repeat debridement and culture on POD 12. 

Her WBC began to settle and became 20.1 x 10⁹/L on POD 14 but then started rising again over the following 2 days, reaching 39.4 on POD 16, and the redness and ulceration of her breasts increased. At this point, the diagnosis of PG was questioned and the dermatology department was consulted. On initial inspection by dermatology, PG was not felt to be the case. However, given the appearance of the wounds on the following day, reassessment by dermatology confirmed PG as the diagnosis. The patient was started on oral prednisone (80 mg/day) and cyclosporine (150 mg in the morning and 200 mg in the evening daily) on POD 15. Following this, she improved significantly. 

On POD 23, she developed what appeared to be cellulitis on her left calf. Over concern of infection, the prednisone was tapered down (it had already been decreased to 50 mg per day on POD 21, then to 40 mg daily on POD 23, 20 mg daily on POD 27, and 10 mg daily on POD 30), and she was started on intravenous immunoglobulin (IVIG) at 1 g/kg infused over 4 hours daily for 3 days. She continued to improve with once-daily dressing changes. The patient was discharged home on POD 34 and continued on prednisone 10 mg daily and cyclosporine 150 mg daily as well as IVIG every 2 weeks (1 g/kg dose infused over 4 hours). 

She was seen for follow-up 2 months after the initial mastopexy. Since her discharge, her dose of cyclosporine had been decreased to 50 mg twice daily. She was still taking 10 mg prednisone daily and having infusions of IVIG (1 g/kg infused over 4 hours) every 2 weeks. Her breasts had made remarkable improvement; the wound edges had begun to contract, the nipples appeared healthy, and there was no evidence of infection. Her wounds were reassessed at postoperative month 3 and again on postoperative month 4 and she continued to improve.

The patient was interested in reconstruction of both breasts; thus, after much discussion and research, the patient underwent surgery 5 months after presentation for debridement and FTSG, harvested from her lower abdomen (Figure 2). A sample from the left breast was collected and sent for pathological analysis, which later confirmed nonspecific, benign ulceration and scarring and no evidence of PG. Postoperatively, the patient was put on prednisone 25 mg daily and cyclosporine 100 mg daily. When seen at 2-week follow-up post-FTSG, it was noted that the skin grafts had partial take on the breasts. There were some open areas, including the right medial breast, left superior breast, and around the edges of the skin grafts, that did not appear to be granulating well. There was no change in management. Home care was continued for dressing changes and she was continued on the same doses of prednisone and cyclosporine. The wound of the donor site in the abdomen was healing well. 

When seen at 5-week follow-up, it was noted that only about 50% of the skin grafts were viable. She appeared to be healing secondarily with reepithelization of the edges. There was significant scarring around her nipple areolar complex. She was still taking 100 mg of cyclosporine daily, and, at this point, she was taking 10 mg of prednisone daily, tapered as directed by dermatology. 

At 3-month follow-up (8 months post presentation), the patient was doing extremely well with excellent wound healing and ongoing scarring to bilateral breasts; there were no signs of infection or open areas (Figure 3). She remained on both 10 mg of prednisone and 100 mg of cyclosporine once daily. 

As of February 2018, there has been no recurrence of the PG.

The authors conducted a literature search on PubMed in June 2016 of all published articles on cases of breast PG that were managed surgically with the application of skin grafts or flaps. This PubMed search was completed using the keywords breast, mastopexy, mammoplasty, and pyoderma gangrenosum. Non-English articles were excluded from the search. This resulted in 80 articles, which yielded 81 cases of confirmed PG in the female breast, 62 of which occurred following surgery, 14 that did not occur following surgery, and 5 where history of surgery before PG development was not specified. The authors found 16 cases of postsurgical PG where skin grafting or skin flaps were used in management of the disease (Table^6-19). All except 1 case²⁰ reported medical immunosuppression in combination with skin grafting/flaps. Nine cases used split-thickness grafting, of which 6 were successful,^6-11 2 failed,^12,13 and 1 had an unspecified outcome.¹⁴ One case successfully used local flaps.¹⁵ Four cases did not specify the type of grafting applied, and of these, 1 was successful,¹⁶ 2 failed,^17,18 and 1 outcome was unknown.¹⁹ Two cases used FTSG, and of these, 1 was successful²⁰ and 1 had an unknown outcome.²¹ In summation, 9 of the cases were successful in their use of skin grafts/flaps, 4 failed, and 3 had an unspecified outcome. Communication to the authors to obtain further details on the cases of unknown outcomes was attempted but unsuccessful. 

Surgery for the application of skin grafts or flaps in the context of PG poses a risk for PG recurrence due to the characteristic pathergy present in the disease.⁵ However, the use of skin grafts or flaps for wound healing in PG may be beneficial in significantly quickening the healing process of the skin wounds. To further speed up the process of treatment and healing, it is important to differentiate PG from necrotizing fasciitis (NF) early in the disease progression. 

Pyoderma gangrenosum of the breast is commonly misdiagnosed as NF. The following key characteristics of PG distinguish it from NF: 

• PG commonly presents bilaterally²²;
• PG spares the area of the nipples²²;
• PG does not respond to antibiotic therapy³; and
• PG tends to worsen following surgical debridement.⁵

Keeping these points in mind, ensuring that PG is on the differential diagnosis when a patient presents with ulcerative lesions following breast surgery can help promote earlier appropriate treatment. 

In this report, the authors presented a case of PG where skin grafting was successful despite the risk of pathergy and the patient being on high doses of immunosuppressants. The patient was middle-aged and healthy, which increased the chance of successful healing of the skin grafts. The skin graft donor site in the abdomen had no occurrence of PG and healed well. The authors were able to use FTSG, a type of grafting that, according to a literature search, has only been used in the management of breast PG twice before.^20,21 The patient had redundant abdominal skin from which the FTSG was harvested, suggesting the use of FTSG as an option to be explored in patients with a similar presentation. The use of FTSG is advantageous because one can primarily close the wound, unlike split-skin grafting, which heals through reepithelization of the dermis and requires dressings.

Overall, this report highlights the use of FTSG in the management of PG, a method that has been successful in this patient case when combined with high-dose steroids and cyclosporine. 

Affiliation: Dalhousie University and Nova Scotia Health Authority, Halifax, Nova Scotia, Canada 

Correspondence: Zeina Asyyed, BSc, 315-1263 Queen Street, B3J 3L4, Halifax, NS, Canada; zn292782@dal.ca 

Disclosure: The authors disclose no financial or other conflicts of interest.