Development of Postsurgical Pyoderma Gangrenosum with New Keloid after Keloid Resection.

ABSTRACT: Keloids are a dermal fibroproliferative disorder and can arise from trauma, acne, vaccination, and herpes zoster. Pyoderma gangrenosum (PG) is a painful ulcerative skin disorder that is associated with neutrophilic dysfunction. However, the pathophysiologies of keloids and PG are not fully understood. The authors encountered an unusual case of a 24-year-old woman who presented with an anterior chest keloid that bore an ulcer. The keloid was resected along with the ulcer, and histology revealed the ulcer to be a neutrophilic PG ulcer. A year after surgery, another ulcer developed in the scar. The ulcer met the PARACELSUS criteria of a postsurgical PG ulcer. After treatment with systemic prednisone and adalimumab for 250 days, the ulcer re-epithelialized. However, relapsed keloids were then observed at the PG site. Corticosteroid taping may be the safest therapy for patients with a history of PG. Conversely, if there is suspicion that a patient is prone to keloid development, diagnostic biopsies and surgical management of PG ulcers should be avoided or conducted with care.

Adjunctive hyperbaric oxygen treatment for challenging pyoderma gangrenosum cases.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, difficult-to-treat neutrophilic ulcerative cutaneous condition that severely impacts those affected. Treatment options for PG are limited, and disease remission is not guaranteed. Hyperbaric oxygen treatment is a potential therapeutic option for treating various ulcerative conditions not frequently utilized for PG. CASE REPORT: We present a case of a patient with treatment-resistant PG who achieved remission with adjunctive HBOT, and then later had difficulty achieving remission without HBOT during a future flare. DISCUSSION: HBOT should be more readily considered as a treatment option for those with PG.

The role of split-thickness skin grafting in the treatment of vasculitic and pyoderma gangrenosum ulcers in a multidisciplinary wound centre.

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.

[Pyoderma gangrenosum after orthopedic conservative treatment]. / Pyoderma gangrenosum après traitement conservateur orthopédique.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Half of the cases are associated with an immune dysfunction and are frequently triggered by pathergy such as a tissular aggression via surgery or burn wounds. A patient with ulcerative colitis presented a PG at the site of an iontophoresis patch for tendinopathy. Treatment in a specialized burn center, corticosteroid therapy and adapted local care contributed to a favourable evolution. PG remains a diagnosis of exclusion and inflammatory phenomena must be differentiated from infectious causes such as necrotizing fasciitis to initiate immunosuppressive treatment. Being rare and difficult to diagnose and to treat as well as associated with potentially severe sequelae, a multidisciplinary team is required for the management of PG.

Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare. Il est, dans la moitié des cas, associé à une maladie dysimmunitaire et il est fréquemment déclenché par un phénomène de pathergie, défini comme une agression tissulaire par une intervention chirurgicale ou encore une brûlure. Une patiente avec une rectocolite ulcéro-hémorragique a développé un PG sur le site d'application d'un patch d'ionophorèse pour une tendinopathie. Un traitement par une corticothérapie, un traitement immunosuppresseur local et des soins locaux adaptés ont permis une évolution favorable. Le PG reste un diagnostic d'exclusion et les phénomènes inflammatoires doivent être différenciés de phénomènes infectieux, comme la fasciite nécrosante, afin d'initier rapidement des immunosuppresseurs. Comme il s'agit d'une pathologie rare avec un diagnostic difficile, que des séquelles peuvent être catastrophiques et qu'un traitement immunosuppresseur complexe doit être instauré, une équipe pluridisciplinaire est requise pour la prise en charge de cette pathologie.

Pyoderma Gangrenosum after Cardiac Surgery.

BACKGROUND: Pyoderma gangrenosum after cardiac surgery is a rare, noninfectious ulcerating skin disease mimicking sternal wound infection. METHODS: A systematic search of literature for pyoderma gangrenosum complicating cases of cardiac surgery was conducted between September 1985 and September 2020 on PubMed and Cochrane databases. A systematic review and detailed overview of clinical presentation, diagnostic, treatment, and outcome is provided. RESULTS: A total of 15 studies enclosing 15 patients suffering from pyoderma gangrenosum following cardiac surgery were identified. Onset of symptoms was observed after a median of 5 days. Patients were predominantly male (81.3%) with a median age of 64 years. Typical clinical presentation mimicked sternal site infection, mainly by means of mediastinitis. Specific signs were rapid progression, erythematous to violaceous color of the wound border, accompanied by unspecific symptoms including fever, malaise, and severe pain. Additionally, pathergy (development of ulcers at the sites of minor cutaneous trauma) was reported frequently. Biopsy is mandatory with a cutaneous neutrophilic inflammation confirming the diagnosis. Initial treatment mostly (75.0% of reported cases) was misled, addressing suspicion of surgical site infection. After correct diagnosis, the treatment was switched to an immunosuppressive therapy. Full sternal wound closure took between 5 weeks and 5 months. Reported case mortality was 12.5% in actually low-risk surgeries. CONCLUSION: Despite pyoderma gangrenosum has typical signs, it remains an exclusion diagnosis. The treatment is completely opposite to the main differential diagnosis-the typical surgical site infection. Knowledge about diagnosis and treatment is essential in the context of avoiding fatal mistreatment.

Pyoderma gangrenosum and dehydrated human amnion/chorion membrane: a potential tool for an orphan disease.

OBJECTIVE: Pyoderma gangrenosum (PG) is an often-misdiagnosed, painful, inflammatory and ulcerative skin disorder. It is an orphan disease, where standard wound treatments such as sharp surgical debridement are contraindicated. This retrospective case series sought to evaluate the application of dehydrated human amnion/chorion membrane (DHACM) as a skin substitute in cases that were refractory to a range of standard-of-care techniques. METHOD: This retrospective case series involved wounds which failed to close with standard escalating treatments, including anti-inflammatory and immunosuppressive therapies. Subjects were transitioned to DHACM and wound sizes were monitored until closure. RESULTS: Wounds (n=5) for all three subjects had stalled with standard therapies for at least 2.5 months but responded quickly to routinely applied DHACM treatments, and closure was achieved in each case. CONCLUSION: This retrospective pilot case series examined the use of DHACM as an alternative wound treatment for PG patients failing standard therapies. DHACM treatments re-initiated the trajectory towards wound closure for each stalled PG ulcer. The results suggest a treatment algorithm starting with early recognition, wound closure via treatment escalation, and lastly a gradual reduction in treatment for durable closure. DHACM treatment should be formally evaluated as an adjunct to PG ulcers that have remained refractory to more commonly used immunomodulating therapies.

Characterization of Legal Claims Involving Patients with Pyoderma Gangrenosum.

OBJECTIVE: To review the litigation literature related to patients with pyoderma gangrenosum (PG) to characterize the legal issues they face. DATA SOURCES: Data sources include law databases Casetext and Lexis Nexis. STUDY SELECTION: All disability and medical liability claims directly involving patients with PG were included. DATA EXTRACTION: Data extraction came directly from Casetext and Lexis Nexis. All cases extracted came from September 1965 to December 2020. This resulted in 32 cases, 18 of which were excluded because they did not mention the plaintiff having a diagnosis of PG (n = 14). DATA SYNTHESIS: The review found a total of six medical liability cases in which adverse health outcomes were reported, including prolonged suffering (2/6 cases), unnecessary treatments or procedures (2/6), exacerbation of disease (1/6 cases), and permanent scarring (1/6 cases). Despite this, two plaintiffs won their case and only one of them received any monetary award. Similarly, of the eight disability claims in the review, four were ruled in the favor of the plaintiffs and only two resulted in immediate awarding of benefits. Half of medical liability cases occurred in correctional facilities after the denial of appropriate care. CONCLUSIONS: The findings demonstrate a need for access to specialty care in incarcerated populations. Cases that occurred in a hospital setting also stress the importance of initiatives such as telemedicine to efficiently increase access to care in a cost-effective manner. Further, PG has been recognized as a severe impairment in disability claims despite patients being denied because they were deemed able to perform other work.

Onset of Pyoderma Gangrenosum in Patients on Biologic Therapies: A Systematic Review.

OBJECTIVE: To summarize clinical outcomes of paradoxical pyoderma gangrenosum (PG) onset in patients on biologic therapy. METHODS: The authors conducted MEDLINE and EMBASE searches using PRISMA guidelines to include 57 patients (23 reports). RESULTS: Of the included patients, 71.9% (n = 41/57) noted PG onset after initiating rituximab, 21.1% (n = 12/57) noted tumor necrosis factor α (TNF-α) inhibitors, 5.3% (n = 3/57) reported interleukin 17A inhibitors, and 1.8% (n = 1/57) reported cytotoxic T-lymphocyte-associated protein 4 antibodies. The majority of patients (94.3%) discontinued biologic use. The most common medications used to resolve rituximab-associated PG were intravenous immunoglobulins, oral corticosteroids, and antibiotics, with an average resolution time of 3.3 months. Complete resolution of PG in TNF-α-associated cases occurred within an average of 2.2 months after switching to another TNF-α inhibitor (n = 1), an interleukin 12/23 inhibitor (n = 2), or treatment with systemic corticosteroids and cyclosporine (n = 3), systemic corticosteroids alone (n = 1), or cyclosporine alone (n = 1). CONCLUSIONS: Further investigations are warranted to determine whether PG onset is associated with underlying comorbidities, the use of biologic agents, or a synergistic effect. Nevertheless, PG may develop in patients on rituximab or TNF-α inhibitors, suggesting the need to monitor and treat such adverse effects.

Neutrophilic Dermatoses in a Clinical Practice of Wound Care Professionals.

ABSTRACT: Diagnosing and treating neutrophilic dermatoses (NDs) in clinical practice can be challenging because of various presentations and stubborn treatment responses. Establishing a diagnosis is necessary, though, because many NDs are associated with underlying conditions, including malignancy. In this article, the authors provide information about Sweet syndrome, pyoderma gangrenosum, and other NDs and describe their clinical presentation, pathophysiology, diagnostic criteria, and associated conditions. The authors also present a case report describing the coexistence of two NDs and hidradenitis suppurativa in one patient and review the treatment modalities for those conditions.

[Ulcerative colitis complicated by pyoderma gangrenosum and multiple aseptic abscesses].

A woman in her 30s was diagnosed with ulcerative colitis (UC) 4 years ago and treated with tacrolimus, azathioprine, and prednisolone 5mg (PSL). Skin ulcers appeared on the right lower leg during the course of treatment, diagnosed as pyoderma gangrenosum (PG). The patient initially improved with an increased PSL and infliximab dose, but then developed multiple skin ulcers and folliculitis throughout her body. She was transferred to our hospital for PG exacerbation treatment. She developed fever after transfer and contrast-enhanced computed tomography showed multiple abscesses in the lungs and kidneys. PSL was decreased and infliximab was discontinued. Antibiotic therapy and granulocyte/monocyte apheresis (GMA) were started. Fever persisted even after antibiotic treatment, and her general condition did not improve. A right renal abscess puncture was performed. Pus was sterile. A sterile abscess associated with PG was suspected. The PSL dose was increased to 1mg/kg and infliximab restarted. Thereafter, the patient's general condition improved, and both lung and renal abscesses contracted. Skin ulcer epithelialization was also observed. Abdominal symptoms were mild during the course of the disease, and colonoscopy showed only a localized ulcerative lesion in the rectum. The patient was later transferred to the department of dermatology at our hospital for PG treatment. Aseptic abscesses are caused by neutrophil infiltration without infection and have been reported to be associated with neutrophilic dermatosis and inflammatory bowel disease. UC-associated aseptic abscess is rare. This is only the sixth case in Japan. Aseptic abscesses can occur in various sites, including subcutaneous and deep organs, but this is the first kidney abscess case. In previous reports, PSL, infliximab, colchicine, and infliximab+GMA were used for aseptic abscesses associated with UC. They all showed abscess reduction. Aseptic abscesses associated with PG should be considered if abscess lesions occur during the course of UC, and a treatment strategy including enhanced immunosuppression should be considered.

The role of negative pressure wound therapy (NPWT) on the treatment of pyoderma gangrenosum: A systematic review and personal experience.

Pyoderma gangrenosum (PG) is a rare painful ulcerative neutrophilic inflammatory skin disease, necessitating a high level of diagnostic suspicion associated with appropriate treatment to avoid progression. Negative pressure wound therapy (NPWT) has been efficiently used in the treatment of different types of wounds. However, the role of NPWT in the management of PG is still controversial, due to the risk of the pathergy phenomenon. In this article, we conducted a systematic review (according to the PRISMA guidelines) on the use of NPWT in the treatment of PG, and we report our personal experience with two patients treated with this device. The result of the review showed that articles on the topic are, in their entirety, of low levels of evidence, such as case series, case reports, and reviews. Improvement in wound healing with the use of NPWT was observed in 85.1% of the patients studied. Besides, a significant association between improvement in wound healing with NPWT and immunosuppressive therapy was observed. Regarding the cases reported here, both showed good outcomes with the use of NPWT and skin graft during the treatment of PG injuries. Due to the rarity of PG, there is a scarcity of studies with robust evidence for standardization and comparison between treatments, which consequently makes it difficult to select therapeutic options. However, based on this systematic review and reported cases, we consider NPWT a safe option for adjuvant treatment of wounds caused by PG if combined with systemic immunosuppression, which plays a key role in greater chances of successful treatment. This approach should be recommended, whenever possible, associated with skin grafting to accelerate wound closure. The role of negative pressure wound therapy (NPWT) on the treatment of pyoderma gangrenosum: a systematic review and personal experience.

A Unified Concept of Acne in the PAPA Spectrum Disorders.

BACKGROUND: Acne is a common condition. Sometimes acne or acne-like features are a part of autoinflammatory diseases such as PAPA spectrum disorders. Our objective wasto determine whether acne in PAPA spectrum disorders has specific phenotypic traits. SUMMARY: This is aliterature review on reported cases of patients with PAPA, PAMI, PASH, PAPASH, PsAPASH, PAC or PASS syndrome. Acne was classified into four categories: moderate, severe, nodulocystic, acne fulminans.One hundred patients were included. Age of onset of acne and topography were the same as in acne vulgaris. Acne's phenotype was at least severe (including acne fulminans, nodulocystic and severe) in 83% of cases. Genetic mutations or variants with potential pathological significance were reported in 49 patients. The addition of isotretinoin to biotherapies was required to control acne in several patients.

Pyoderma Gangrenosum Occurring in the Setting of Hypercortisolism Associated With Adrenocortical Adenoma: A Pathophysiological Paradox.

Pyoderma gangrenosum (PG) is a challenging, rare, ulcerating skin disease characterized by neutrophilic abundance and absence of infection, often associated with systemic diseases. We present a 25-year old previously healthy female with a 1.5-year history of treatment refractory PG. Features of Cushing’s syndrome such as facial plethora, striae, and lipodystrophy were noted on exam, which prompted several studies that ultimately revealed an adrenal adenoma. Following surgical excision of the adenoma, symptoms rapidly resolved and systemic immunosuppressants were discontinued. This rare case highlights the importance that adrenal adenoma and resultant Cushing’s syndrome may be a driver of PG despite the pathophysiologic paradox. J Drugs Dermatol. 2021;20(1):95-97. doi:10.36849/JDD.5566.

Minced Skin Grafting: A Minimally Invasive and Low-Cost Procedure to Treat Pyoderma Gangrenosum.

ABSTRACT: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that usually presents as a painful ulcer with erythematous and undermined borders and is often characterized by pathergy. Although this condition precludes a surgical approach, in cases resistant to treatment with immunosuppressive agents, skin grafts and amputation are considered last resorts. Both expose the patient to the risk of developing new lesions. A minimally invasive autologous graft, "minced micrografts," is a possible alternative to speed up the healing process, reducing the risk of pathergy.This procedure was implemented in a 28-year-old man with PG who was prescribed prednisone and dapsone and who had a large ulcer on the arm and armpit that had persisted for 4 months and was undergoing slow re-epithelialization. A sample of finely minced skin previously taken from the clavicular region suspended in a hydrogel was spread on the wound bed in a proportion of less than 1:6 with respect to the receiving site. Seven days later, initial signs of re-epithelialization appeared, and the wound healed in 3 months.In patients with PG, the minced micrograft method could facilitate ulcer healing by releasing cytokines, chemokines, and growth factors, thus promoting granulation tissue formation, neoangiogenesis. Because this method does not require special equipment or complex surgical techniques and is very low cost, it should be an integral part of the arsenal of procedures aimed at improving the quality of life of patients with PG.

Case Report of Two Patients With COVID-19 and Sacral Pressure Injuries Associated with Pyoderma Gangrenosum.

ABSTRACT: During the COVID-19 pandemic, an increasing number of patients have been admitted to the ICU with severe respiratory complications requiring prolonged supine positioning. Recently, many case reports have been published regarding dermatologic manifestations associated with COVID-19. However, there is little information about the clinical features of these manifestations. Pyoderma gangrenosum (PG) is an ulcerative noninfectious inflammatory disease of the skin. In at least 50% of the cases, the etiology is unknown. Nevertheless, PG is associated with many systemic diseases. In this article, the authors report two critically ill patients with COVID-19 who developed sacral ulcers during their recovery in the ICU. These ulcers had an atypical course and were exacerbated by surgical debridements. Accordingly, providers suspected PG, which was confirmed by the clinical evolution of the ulcers and biopsies taken from the wounds. To the best of the authors' knowledge, no previous articles have reported sacral pressure injuries associated with PG in patients with COVID-19. Providers should suspect PG in patients with COVID-19 who develop nonhealing pressure injuries.

Pyoderma Gangrenosum: A Rare Disease With Dire Consequences in Facial Aesthetic Surgery Patients.

Pyoderma gangrenosum (PG) is a rare, inflammatory dermatologic condition characterized by painful cutaneous ulcerations. Herein, we describe the third documented case of PG arising in an elective plastic surgery patient who had undergone an otherwise uncomplicated facelift. We describe the course of her diagnosis and management of PG, which involved her face and neck and then progressed to her lower extremities. Although the etiology remains unknown, PG often arises in a host with another autoimmune disease. In the case described, the patient was diagnosed with an immunoglobulin A gammopathy shortly after she developed PG. Following the case report, the pathogenesis, diagnosis, and treatment strategy of PG is briefly reviewed. Level of Evidence: 5.

Pyoderma gangrenosum in an ulcerative colitis patient during treatment with vedolizumab responded favorably to adsorptive granulocyte and monocyte apheresis.

Pyoderma gangrenosum (PG) is an extra-intestinal skin lesion in inflammatory bowel disease (IBD) as is erythema nodosum. Vedolizumab (VED) is a monoclonal antibody that targets α4ß7 integrin and has an intestinal selective mechanism. Despite good therapeutic effects on colitis, the effect on extra-intestinal manifestations (EIMs) remains unclear. Here we report a case of ulcerative colitis complicated by PG during treatment with VED, which was successfully treated with prednisolone in combination with adsorptive granulocyte and monocyte apheresis (GMA). The patient was a 50-year-old woman with a past medical history of extensive ulcerative colitis managed by golimumab (GLM). She developed flare symptoms due to loss of response to GLM, and treatment was switched to VED. Her gastrointestinal symptoms were improved with VED treatment with less frequent bowel movements. However, infiltrative erythema with pain appeared on the right lower leg and right knee, and expanded and gradually ulcerated. Her skin lesions were treated with corticosteroid, but showed poor improvement. Therefore, granulocyte and monocyte apheresis (GMA) treatment was administered in combination with prednisolone. After 3 months, the ulcer gradually improved, and at the time of this writing, the eruptions were nearly replaced by epithelial tissue. This case study showed that patients with UC and EIMS may respond well to combination therapy of VED and GMA. GMA has a very favorable safety profile. On the other hand, the causal connection between VED and PG is still unclear. We believe that a combination therapy involving VED and GMA in IBD patients with EIMs warrants consideration.

Pyoderma gangrenosum after cardiac surgery.

Pyoderma gangrenosum (PG) can mimic early postoperative sternal wound infections. Steroid therapy is the only method of treatment for this condition, which recognizes the fact that steroids can lead to immunosuppression and potentially inhibit healing after major surgery.

Casos de pioderma gangrenoso en pacientes con quemaduras: revisión de la literatura.

OBJECTIVE: Perform a literature review of case studies on pyoderma gangrenosum (PG) associated with burns, and compare the results with a clinical case study on PG in a patient with wounds, published by the authors in this same article. METHOD: PubMed, ScienceDirect and ClinicalKey were searched for articles between June 2006 and July 2020. Of 831 articles, only 43 met the inclusion criteria. Nine articles, with 17 case studies, were included. The authors' case study was also evaluated (C18). RESULTS: Of the 18 case studies, 50% were female. In 55% cases, the most affected area was the lower limb. The most frequently applied treatment was corticoids (88.9% of cases). CONCLUSION: PG was present mostly on healthy areas, already healed. PG often develops on the lower limb, but can also be present in any anatomic area. It can emerge at any age, even in underaged patients.

OBJETIVO: Realizar una revisión de la literatura de casos de pioderma gangrenoso (PG) asociados con quemaduras, y comparar los resultados con un caso clínico de PG en un paciente con quemaduras, presentado por los autores en este mismo artículo. MÉTODO: Se revisó la literatura en las bases de datos PubMed, ScienceDirect y ClinicalKey, entre junio de 2006 y julio de 2020. De los 831 artículos encontrados, sólo 43 cumplieron con los criterios de inclusión. Nueve artículos, con 17 casos de estudio, fueron seleccionados. Se evaluó, además, el caso de estudio realizado por los autores (C18). RESULTADOS: De los 18 casos totales, 50% fue mujer y, el restante 50%, hombre. En el 55% de los casos, las zonas con lesiones más afectadas fueron las del miembro inferior. El tratamiento más frecuentemente empleado fue con corticoides, en 88,9% de los casos. Hubo similitudes entre los casos estudiados. CONCLUSIÓN: En lesiones traumáticas, como las quemaduras, el PG se presenta mayoritariamente en áreas sanas, ya cicatrizadas. Ocurre, con frecuencia, en los miembros inferiores, pero puede aparecer en cualquier área anatómica. Se presenta a cualquier edad, incluso en menores de edad, con mayor frecuencia en la quinta década de la vida. CONFLICTO DE INTERÉS: Ninguno.

ARTERIAL INSUFFICIENCIES: Hyperbaric Oxygen Therapy for Selected Problem Wounds.

The use of hyperbaric oxygen (HBO2) for the treatment of selected problem wounds has focused almost entirely on the diabetic foot ulcer (DFU) in recent years. The prevalence of DFUs in today's patient population and the reimbursement available for the treatment of DFUs have given it priority status in discussions about problem wounds, but there are sound fundamental reasons why additional oxygen may have benefits in the treatment of non-DFU wounds.