Postsurgical Pyoderma Gangrenosum Complicating Reduction Mammaplasty
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- Grillo, M.A., Cavalheiro, T.T., da Silva Mulazani, M. et al. Aesth Plast Surg (2012) 36: 1347. doi:10.1007/s00266-012-9981-3
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Reduction mammaplasty is one of the most common surgeries performed by plastic surgeons. It relieves back and neck pain and improves the aesthetic contour of the ptotic breast. Postsurgical pyoderma gangrenosum (PSPG) is an unusual inflammatory disorder leading to rapidly progressive skin necrosis that can occur after any surgical procedure. The skin lesions have the characteristic appearance of ulcers with a purple-colored border and erythematous halo. Clinically, the patient has a low fever and severe local pain. In the majority of cases this disease is misdiagnosed as severe infection leading to improper debridement, exacerbating the problem. The mainstay of therapy for PSPG is still nonoperative and focuses on immunosuppressive medications and local wound care, which allows healing in the majority of the cases. It is important for plastic surgeons and infectologists to be cognizant of this entity, as a delay in diagnosis and management can be life-threatening and lead to considerable tissue loss and disfigurement of the breast. The authors report a case of reduction mammaplasty complicated with PSPG and its treatment.
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KeywordsBreast reductionPostsurgical pyoderma gangrenosumPathergyMammaplasty/adverse effects
Postsurgical pyoderma gangrenosum (PSPG), also called postoperative pyoderma gangrenosum, necrotizing ulceration, or postoperative progressive gangrene of Cullen, is a rare complication of surgical procedures characterized by painful skin lesions with rapidly progressing necrosis. Primarily it is not associated with surgical infection but its similarity delays the diagnosis and treatment.
Reduction mammaplasty is a very common procedure. The ISAPS (International Society of Aesthetic Plastic Surgery) international survey on aesthetic/cosmetic procedures performed in 2009 showed that there were 509,396 breast reductions worldwide. It is the sixth most common plastic surgery procedure. In 2008, the American Society of Plastic Surgeons reported that over 79,400 women underwent breast reduction surgery in the US , and the Datafolha Research Institute showed that there were 67,000 reduction mammaplasties performed in Brazil .
We report a case of PSPG complicating a breast reduction and discuss some important topics about its diagnosis and treatment.
Antimicrobial therapy was changed empirically to ampicillin and sulbactam. Despite treatment, there was an impressive worsening of the lesions’ appearance. Blood and skin cultures for anaerobic and aerobic bacteria, mycobacteria, and fungi were persistently negative. The patient was surgically manipulated again, 4 days after the first debridement, and it was decided to switch to tigecycline empirically. Histopathologic examination of the skin lesion revealed extensive ulceration, suppurative inflammation with skin abscess, and neutrophil infiltration with hypodermic extension to the breast tissue suggesting pyoderma gangrenosum (PG).
Based on clinical and pathological findings and on the lack of response to large-spectrum antibiotic therapy, treatment for PG (prednisone 60 mg/day) was initiated on the sixth day after admission. Despite the potential risk of a hepatitis B flare-up at this point, it was decided not to use lamivudine or other prophylactic treatment. Other side diseases were considered, but all laboratory tests pointed only to hepatitis B as the probable trigger. After the start of prednisone there was great improvement clinically and on the breasts with reduction of swelling and progression of ulcers and consequently pain relief.
Postoperative or PSPG, also known as postoperative progressive gangrene of Cullen, was first descried by Cullen in 1924 . In 1930, PG was described as an uncommon, ulcerative, cutaneous condition . This skin disorder was termed PG because the authors believed that the spreading of the lesion resulting in gangrene was caused by a serious streptococcal infection .
Over 500,000 breast reductions were performed around the world in 2008. Minor complications such as small hematomas, wound breakdown, fat necrosis, and local wound infection are common and do not require readmission to the hospital . Although rare, PG has been reported following 22 breast reductions and pexies, 7 breast reconstructions, and 2 breast augmentations [1, 3–25].
PG has been classified as a neutrophilic dermatosis as it exhibits intense dermal inflammatory infiltrates composed of neutrophils . It is believed to result from a misdirected immune system response to injury. The pathogenesis of PG is poorly understood, but neutrophil dysfunction (i.e., defects in chemotaxis or hyperreactivity) has been suggested. Furthermore, interleukin-8 (IL-8), a potent leukocyte chemotactic agent, has been shown to be overexpressed in PG ulcers .
Histopathologic findings are not specific but they are useful in making the right diagnosis in combination with clinical evolution. Skin biopsy specimens taken from the necrotic, undermined ulcer border of PG reveal mixed cellular inflammation with neutrophil predominance . The immunological reaction determines the capillary dilatation and subsequent cutaneous rash. It starts with a chain reaction, with intense neutrophil migration to the reaction site located in the basal layer of the skin, followed by a great production of collagenase. This enzyme destroys collagen and the capillary network that nourishes the skin’s basal layer. With links broken, the skin breaks down and necrosis begins. In the presence of necrotic tissue, the body increases the local number of neutrophils and macrophages to digest and complete cleaning of the wound. Neutrophils in larger quantities produce more collagenase, which destroys more cutaneous tissue and perpetuates the cycle. Skin atrophy creates a thin keratinized epithelial layer that forms bubbles containing the remains of digested and liquefied dermis, resulting in a dark blue or purple color. The bubble quickly bursts and the enzymes are spread across the wound, continuing the digestive process resulting in ulceration. This reaction, by contiguity, affects the surrounding normal skin, which causes cutaneous rash and again the destructive cycle repeats itself indefinitely . Clinically, it appears as erythematous pustules which become boggy plaques with purple-colored margins and an erythematous halo. There is then rapid progression of cutaneous ulceration and spreading necrosis (Fig. 2). Normally, the patient is pyrexic and complains of disproportionate pain [1, 27].
Proposed diagnostic criteria of classic ulcerative PG (diagnosis requires both major criteria and at least two minor criteria)
1. Rapid progression of a painful, necrolytic cutaneous ulcer with an irregular, violaceous, and undermined border
2. Other causes of cutaneous ulceration have been excluded
1. History suggestive of pathergy or clinical finding of cribriform scarring
2. Systemic diseases associated with PG
3. Histopathologic findings (sterile dermal neutrophilia, mixed inflammation, lymphocytic vasculitis)
4. Treatment response (rapid response to systemic steroid treatment)
Differential diagnosis of PG
Vascular occlusion or stasis
Antiphospholipid-antibody syndrome; livedoid vasculopathy; venous stasis ulceration; Klippel-Trénaunay-Weber syndrome; small-vessel-occlusive arterial disease; type-1 cryoglobulinemia
Wegener granulomatosis; polyarteritis nodosa; cryoglobulinemic vasculitis (mixed cryoglobulinemia); Takayasu arteritis; leukocytoclastic vasculitis
Cutaneous involvement by malignant process
Angiocentric T-cell lymphoma; anaplastic large-cell T-cell lymphoma; mycosis fungoides bullosa; unspecified lymphoma; leukemia cutis; histiocytosis X (Langerhans cell histiocytosis)
Primary cutaneous infection
Sporotrichosis; Aspergillosis; Cryptococcosis; Herpes simplex type 2 virus; cutaneous tuberculosis; Amebiasis cutis; zygomycosis; Penicillium marneffei
Drug-induced and exogenous tissue injury
Munchausen syndrome and factitious disorder; hydroa-induced ulceration; bromoderma; contact vulvitis; drug-induced lupus; loxoscelism (brown recluse spider bite); injection drug abuse with secondary infection
Other inflammatory disorders
Cutaneous Crohn disease; ulcerative necrobiosis lipoidica
Often, the similarity to an infectious etiology delays the diagnosis. Sixty to eighty percent of patients with PG present with a history of other inflammatory disorders, with inflammatory bowel disease being the most common. Other associated disorders include polyarthritis, monoclonal gammopathy, HIV infection, malignant hematologic neoplasms such as leukemia, lupus, chronic urinary diseases, pemphigus, and hypothyroidism [3, 5, 27]. It may also be associated with chronic hepatitis, although this event is rarely described in the literature. This case report presents the first publication of a patient with chronic hepatitis B who had reduction mammaplasty and developed PSPG.
In 10–20 % of cases, PG is idiopathic . In breast surgery, initial erythema, followed by blistering and necrosis, may begin anywhere from 4 to 14 days after surgery. Almost 25 % of the clinical cases reported a history of pathergy, and in all cases the problem was initially thought to be the result of infection. The patients normally undergo operative debridement and systemic antibiotics, neither of which halts progression of the disease . In the first reported case of PG developed after breast reduction, the patient had several operations over 28 months before diagnosis .
The treatment for patients who have a severe rapid evolution is the use of a systemic agent (corticosteroids 40–120 mg daily). Immunosuppressive agents like methotrexate, cyclophosphamide, and immunoglobulin have been used for patients who do not respond to other therapies or who develop steroid-related side effects . Other drugs used are azathioprine, dapsone, mercaptopurine, sulphasalazine, sulphapyridine, thalidomide, and cyclosporine .
Immediately after the initiation of corticosteroid therapy (60 mg daily), the patient had a dramatic recovery in her clinical status and local wound appearance, with pain relief and erythema/edema involution. Immunosuppressive therapy in patients with chronic hepatitis B is controversial because it may trigger acute exacerbation of hepatitis. As our patient had a low viral load, AgHBe was negative, without an increase in transaminase levels prior the treatment, it was decided not to start prophylaxis with lamivudine. It is essential to monitor liver function tests in such patients.
Some reports have shown both early and late failure of wound coverage with a skin graft or flap at debridement . Grafting should be undertaken only in patients who have no surgical evidence of active disease and are receiving adequate immunosuppressive therapy . Long-term (1 year or more) postoperative surveillance is necessary because late failure of the graft or flap can occur during this period . Some authors claim that operative therapy is best avoided . As the tissue defect of our patient was very large in both breasts and the disease was very aggressive, and with the possibility of pathergy occurrence at the skin graft or flap donor site, the clinical team decided to continue with local wound care and hyperbaric oxygen therapy, which also has been reported to be effective .
PSPG represents a specific entity. It shares some clinical aspects of PG, but has its own features. First, the onset of PSPG follows a sequence. After apparently normal evolution of scar formation following a surgical procedure, the scar presents with many small dehiscences, which will progressively coalesce into some larger areas of wound ulceration, with no visible granulation tissue. Second, the period between surgery and the beginning of symptoms varies from 4 days to sometimes weeks. The process will evolve well beyond what would be expected for the initial wound that was created by surgery (pathergy), with no self-limitation. The skin ulcerations become larger despite local treatment or antibiotics. Third, PSPG can affect any anatomical location but it rarely affects the nipple–areola complex. Fourth, a dramatic response to immunomodulatory drugs will be observed [4, 7, 16, 18, 19, 21, 23–25].
Finally we are alerting all plastic surgeons to be aware of this disease because its occurrence may follow all surgical procedures. It is especially distressing in breast surgery where the disease’s aggressiveness presents a serious compromise to the final result.
Conflict of interest
The authors have no conflicts of interest to disclose.