Acute postoperative pyoderma gangrenosum case and review of literature identifying chest wall predominance and no recurrence following skin grafts
Authors: Maryam Liaqat, Ashley N. Elsensohn, C. David Hansen, Julie A. Maughan, Marta J. Petersen
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Acute postoperative pyoderma gangrenosum (PPG) is a rare ulcerative cutaneous disease often misdiagnosed as a postoperative wound infection. The etiology of PG is unknown, although abnormal neutrophil chemotaxis has been implicated.1Adachi Y. Kindzelskii A.L. Cookingham G. Shaya S. Moore E.C. Todd 3rd, R.F. et al.Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum.J Invest Dermatol. 1998; 111: 259-268Crossref PubMed Scopus (130) Google Scholar Most recently, a novel mutation in PSTPIP1, which encodes a cytoskeletal adaptor protein that inhibits macrophage function, was identified in a patient with aggressive PG. The mutated protein induced filopodia formation in macrophages and led to increased matrix degradation.2Starnes T.W. Bennin D.A. Bing X. Eickhoff J.C. Grahf D.C. Bellak J.M. et al.The F-bar protein PSTPIP1 controls extracellular matrix degradation and filopodia formation in macrophages.Blood. 2014; 123: 2703-2714Crossref PubMed Scopus (59) Google Scholar We present a patient seen at our institution and key findings from analysis of 90 episodes of PPG in 87 patients from the literature.A 30-year-old woman with a history of hypogammaglobulinemia presented with bilateral breast wound breakdown 6 days after breast reduction surgery. She was febrile with a white blood cell count of 18.8 (10³/μL). Cultures from the right breast wounds were positive for Pseudomonas aeruginosa. She was treated with multiple antibiotics and repeated surgical debridement without improvement. On examination, there was necrotic eschar overlying bilateral breast ulcers with surrounding superficial bullae and intense erythema. A perilesional biopsy demonstrated a dense neutrophilic dermal infiltrate without evidence of vasculitis or infection; special stains for bacteria were negative. PPG was diagnosed and the patient was started on methylprednisolone 60 mg 4 times daily and cyclosporine 300 mg daily with improvement noted within 48 hours. The patient was discharged on a prednisone taper and cyclosporine 300 mg daily. Complete healing occurred in 3 months.The patient elected to undergo bilateral transverse rectus abdominis myocutaneous flap breast reconstruction surgery 6 months after her initial surgery. Preoperatively, she continued cyclosporine 300 mg daily. Again, diffuse wound breakdown occurred 5 days postoperatively. Cyclosporine was continued and the patient was started on methylprednisolone 30 mg every 6 hours with rapid improvement and healing 4 months later.In review of 76 published articles, 106 PPG cases were identified. Of these, 87 patients undergoing 90 procedures met criteria for developing wound breakdown within 21 days following surgery, consistent with acute PPG (Table I), and were included in the analysis. PPG was reported following surgery involving the chest wall in 42 cases (47%) (Fig 1). Nineteen (21%) cases had a subsequent surgical procedure after initial PPG: 14 skin grafts, 1 femoral bypass, 1 breast reconstruction, 1 breast flap procedure, 1 flap procedure for postsurgical PG of the knee, and 1 total hip repair. PPG did not develop after any skin grafting procedures (14 of 19 cases) postoperatively at either the donor or grafted sites. In contrast, 4 of 5 non–skin graft procedures developed PPG.Table ISummary of patients with postoperative pyoderma gangrenosumVariableNo. (%)Total patients87Prior pyoderma gangrenosum (PG)19 (21) Postoperative12 Spontaneous5 Traumatic2Prior surgeries without PG14 (16)Days to onset after surgery Average7 Range1-21Procedures after postoperative PG19 (21) Skin grafts14 Recurrence of PG0 Other∗Other procedures include femoral bypass, breast reconstruction, total hip repair, flap procedure/skin graft for postsurgical PG of the knee, flap procedure to cover for breast implants.5Recurrence of PG4∗ Other procedures include femoral bypass, breast reconstruction, total hip repair, flap procedure/skin graft for postsurgical PG of the knee, flap procedure to cover for breast implants. Open table in a new tab Our review indicates a higher propensity of PPG following chest wall surgery. Recognizing pathergy as a potential trigger of PPG, we speculate the chest wall predominance of PPG could be related to increased mechanical stress on wounds in this region, similar to the propensity of keloids to occur on the chest. Our review also noted lower recurrence of PPG in skin graft procedures than in other procedures, although the numbers were small. The less invasive nature of skin graft harvesting and placement, compared with more invasive initial surgical procedures, may explain why PG did not occur postoperatively in these cases. Concomitant use of immunosuppressants during skin grafting may have also contributed to lack of PPG seen following skin grafting. Further studies are needed in this area, because dermatologists are often asked to comment on the probability of PG recurrence with subsequent surgeries. To the Editor: Acute postoperative pyoderma gangrenosum (PPG) is a rare ulcerative cutaneous disease often misdiagnosed as a postoperative wound infection. The etiology of PG is unknown, although abnormal neutrophil chemotaxis has been implicated.1Adachi Y. Kindzelskii A.L. Cookingham G. Shaya S. Moore E.C. Todd 3rd, R.F. et al.Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum.J Invest Dermatol. 1998; 111: 259-268Crossref PubMed Scopus (130) Google Scholar Most recently, a novel mutation in PSTPIP1, which encodes a cytoskeletal adaptor protein that inhibits macrophage function, was identified in a patient with aggressive PG. The mutated protein induced filopodia formation in macrophages and led to increased matrix degradation.2Starnes T.W. Bennin D.A. Bing X. Eickhoff J.C. Grahf D.C. Bellak J.M. et al.The F-bar protein PSTPIP1 controls extracellular matrix degradation and filopodia formation in macrophages.Blood. 2014; 123: 2703-2714Crossref PubMed Scopus (59) Google Scholar We present a patient seen at our institution and key findings from analysis of 90 episodes of PPG in 87 patients from the literature. A 30-year-old woman with a history of hypogammaglobulinemia presented with bilateral breast wound breakdown 6 days after breast reduction surgery. She was febrile with a white blood cell count of 18.8 (10³/μL). Cultures from the right breast wounds were positive for Pseudomonas aeruginosa. She was treated with multiple antibiotics and repeated surgical debridement without improvement. On examination, there was necrotic eschar overlying bilateral breast ulcers with surrounding superficial bullae and intense erythema. A perilesional biopsy demonstrated a dense neutrophilic dermal infiltrate without evidence of vasculitis or infection; special stains for bacteria were negative. PPG was diagnosed and the patient was started on methylprednisolone 60 mg 4 times daily and cyclosporine 300 mg daily with improvement noted within 48 hours. The patient was discharged on a prednisone taper and cyclosporine 300 mg daily. Complete healing occurred in 3 months. The patient elected to undergo bilateral transverse rectus abdominis myocutaneous flap breast reconstruction surgery 6 months after her initial surgery. Preoperatively, she continued cyclosporine 300 mg daily. Again, diffuse wound breakdown occurred 5 days postoperatively. Cyclosporine was continued and the patient was started on methylprednisolone 30 mg every 6 hours with rapid improvement and healing 4 months later. In review of 76 published articles, 106 PPG cases were identified. Of these, 87 patients undergoing 90 procedures met criteria for developing wound breakdown within 21 days following surgery, consistent with acute PPG (Table I), and were included in the analysis. PPG was reported following surgery involving the chest wall in 42 cases (47%) (Fig 1). Nineteen (21%) cases had a subsequent surgical procedure after initial PPG: 14 skin grafts, 1 femoral bypass, 1 breast reconstruction, 1 breast flap procedure, 1 flap procedure for postsurgical PG of the knee, and 1 total hip repair. PPG did not develop after any skin grafting procedures (14 of 19 cases) postoperatively at either the donor or grafted sites. In contrast, 4 of 5 non–skin graft procedures developed PPG. Our review indicates a higher propensity of PPG following chest wall surgery. Recognizing pathergy as a potential trigger of PPG, we speculate the chest wall predominance of PPG could be related to increased mechanical stress on wounds in this region, similar to the propensity of keloids to occur on the chest. Our review also noted lower recurrence of PPG in skin graft procedures than in other procedures, although the numbers were small. The less invasive nature of skin graft harvesting and placement, compared with more invasive initial surgical procedures, may explain why PG did not occur postoperatively in these cases. Concomitant use of immunosuppressants during skin grafting may have also contributed to lack of PPG seen following skin grafting. Further studies are needed in this area, because dermatologists are often asked to comment on the probability of PG recurrence with subsequent surgeries.