Fluorescent Imaging as a Component of Diagnosing Pyoderma Gangrenosum: A Case Report.

ABSTRACT: A 64-year-old White woman was admitted to the hospital with complaint of progressive right hip ulceration at the wound site following a total right hip arthroplasty. Initial history and physical examination gave a leading differential diagnosis of pyoderma gangrenosum. Until recently, the exclusion of infection for pyoderma gangrenosum has been largely clinical and supported by cultures/biopsies demonstrating the absence of infection. The MolecuLight i:X (MolecuLight, Toronto, Ontario, Canada) is a novel bedside fluorescent imaging device capable of determining the bacterial burden within a wound in real time. Fluorescent imaging excluded infection at the initial visit, and debridement was avoided. Subsequently, pathergy was avoided as well. The patient was started on topical clobetasol with hypochlorous acid-soaked dressings. She also received 80 mg daily of prednisone and high-dose vitamin D3 (10,000 IU). Recovery was complicated by a deep tunnel along the incisional line at 3 months postdiagnosis, which required slowing of the prednisone taper and the addition of colchicine. Repeat cultures grew Parvimonas, Pseudomonas, and Streptococcus species. Appropriate antibiotics were given. The patient was transitioned from prednisone to adalimumab and started on negative-pressure wound therapy. Negative-pressure wound therapy was discontinued at 5 months, and the wound resolved at 6 months.

[Anakinra therapy in a patient with multifocal pyoderma gangrenosum and hidradenitis suppurativa]. / Anakinra-Therapie eines multifokalen Pyoderma gangraenosum bei einem Patienten mit Hidradenitis suppurativa.

The case of a patient with pyoderma gangrenosum and exacerbation after an intense massage is reported. After different immunosuppressive therapies and former diagnosis of hidradenitis suppurativa in his medical history the patient underwent therapy with anakinra 100 mg per day and showed good improvement of his ulcers.

Altered keratinization and vitamin D metabolism may be key pathogenetic pathways in syndromic hidradenitis suppurativa: a novel whole exome sequencing approach.

BACKGROUND: Diagnosis of pyoderma gangrenosum, acne and hidradenitis suppurativa (PASH) and pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH) patients, in spite of recently identified genetic variations, is just clinical, since most patients do not share the same mutations, and the mutations themselves are not informative of the biological pathways commonly disrupted in these patients. OBJECTIVE: To reveal genetic changes more closely related to PASH and PAPASH etiopathogenesis, identifying novel common pathways involved in these diseases. METHODS: Cohort study on PASH (n = 4) and PAPASH (n = 1) patients conducted using whole exome sequencing (WES) approach and a novel bioinformatic pipeline aimed at discovering potentially candidate genes selected from density mutations and involved in pathways relevant to the disease. RESULTS: WES results showed that patients presented 90 genes carrying mutations with deleterious and/or damage impact: 12 genes were in common among the 5 patients and bared 237 ns ExonVar (54 and 183 in homozygosis and heterozygosis, respectively). In the pathway enrichment analysis, only 10 genes were included, allowing us to retrieve 4 pathways shared by all patients: (1) Vitamin D metabolism, (2) keratinization, (3) formation of the cornified envelope and (4) steroid metabolism. Interestingly, all patients had vitamin D levels lower than normal, with a mean value of 10 ng/mL. CONCLUSION: Our findings, through a novel strategy for analysing the genetic background of syndromic HS patients, suggested that vitamin D metabolism dysfunctions seem to be crucial in PASH and PAPASH pathogenesis. Based on low vitamin D serum levels, its supplementation is envisaged.

Pyoderma gangrenosum after orthopaedic or traumatologic surgery: a systematic revue of the literature.

INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterised by a painful ulceration mimicking infection of the operative site. To this day, there is still no general agreement on the medical and surgical treatment of PG. This systematic review of the literature aims to summarise recent studies about post-operative PG in orthopaedic surgery to improve its medical and surgical management. METHOD: In April 2017, we carried out an exhaustive review of the literature in MEDLINE, PubMed and Cochrane databases. Key words were pyoderma gangrenosum, orthopaedic surgery, and surgical wound infection. We identified 183 articles. After excluding articles reporting idiopathic PG, cases secondary to non-orthopaedic surgery, and cases about other subtypes of dermatosis, 30 studies were identified. We only included articles reporting PG after orthopaedic or trauma surgery. RESULTS: Thirty-one cases of PG have been reported, 58% (18) of which were in women, whose mean age was 56.5 years. Clinical signs were constant, the most frequently affected site was lower limbs [77.4% (24)] and delay of symptom onset was two to 17 days. Systemic corticosteroid therapy was systematic, polyvalent immunoglobulins were used in two cases and immunosuppressive drugs in one. Negative pressure therapy was used in seven cases and hyperbaric oxygen in three. DISCUSSION: Delayed diagnosis leads to one or more surgical revisions, which could have been avoided by using early and adapted medical treatment. Early onset of a painful and infected ulcer at the operating site in a patient at risk of PG is an indicator that dermatologist advice is recommended before surgical debridement. Surgical revision, outside the inflammatory phase and/or covered by a systemic corticosteroid therapy, does not lead to PG relapse. LEVEL OF EVIDENCE: IV: Systematic revue of the literature.

Inpatient management of pyoderma gangrenosum: treatments, outcomes, and clinical implications.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare, ulcerative neutrophilic skin condition of unknown etiology. The disease continues to pose therapeutic challenges, with ongoing controversy regarding the role of surgery. METHODS: A retrospective medical records review was conducted for 29 patients who were diagnosed and treated for PG at an Australian tertiary center over 10 years, from 1 January 2000 to 31 December 2010. RESULTS: A total of 29 patients had a diagnosis of PG, with a total of 35 admissions. Nearly all patients had immunosuppressant therapy and 10 (35%) patients underwent surgery. Eight (28%) received hyperbaric oxygen therapy. Complications secondary to medical therapy occurred in 23 (66%) of admissions, with the commonest being poor blood sugar control in patients with diabetes (n = 6, 17%) and steroid-induced diabetes (n = 5, 14%). At discharge, 21 (72%) patients' ulcers had improved and there were 4 (14%) inpatient deaths. At 6 months, 3 of 10 cases with available follow-up showed complete ulcer healing. Most of the patients (n = 8, 80%) who underwent combined medical and surgical therapy had ulcers that had either completely healed or improved at 6 months after discharge. All 3 patients who underwent split skin grafting under immunosuppressive cover (with 2 having hyperbaric oxygen therapy) had no postoperative graft failure or pathergy. CONCLUSIONS: Pyoderma gangrenosum remains a therapeutic challenge, with significant complications and morbidity from long-term medical treatment. Surgery should be considered in conjunction with combined hyperbaric and immunosuppressive therapy once the disease is quiescent, to reduce disease-related comorbidity as well as the consequent adverse effects of long-term immunosuppressant therapy.

Pioderma gangrenoso: presentación de un caso / Gangrenous pyoderma: case presentation

El pioderma gangrenoso es una enfermedad de etiología desconocida, con variedad de manifestaciones clínicas especialmente cutáneas, de difícil diagnóstico, de evolución crónica, con exacerbaciones y remisiones frecuentes, en la cual no existe un tratamiento de elección efectivo, por lo que su respuesta a la terapia es muy variable. Se presentó el caso de una mujer, mestiza, de 64 años de edad, con antecedentes patológicos personales de gastritis, hipertensión arterial y cardiopatía isquémica, con un año de evolución de una lesión ulcerada en primer artejo de pie derecho, con fracasos terapéuticos anteriores al diagnóstico. Se le realizaron estudios hematológicos e histopatológico, confirmándose un pioderma gangrenoso, que respondió favorablemente a la terapia con prednisona, dapsona y oxigenación hiperbárica(AU)

The gangrenous pyoderma is a disease of unknown etiology, with various clinical manifestations, especially cutaneous ones, of difficult diagnosis, chronic evolution and frequent exacerbations and remissions. There is not an effective elective treatment, so the answer to therapy is very variable. We presented the case of a female patient, mestiza, aged 64 years, with personal pathological antecedents of gastritis, arterial hypertension and ischemic cardiopathy, showing a year of evolution ulcerous lesion on the first joint of the right foot with therapeutic failures before the diagnosis. We ordered hematologic and histopathologic studies confirming a gangrenous pyoderma that favorably answered to the therapy with prednisone, dapsone and hyperbaric oxygenation(AU)

Multiple skin ulcers due to Serratia marcescens in a immunocompetent patient.

Serratia marcescens is a species of gram negative bacillus, classified as a member of the Enterobacteriaceae, mainly involved in opportunistic infections, particulary in the hospital environment. Cutaneous infections have rarely reported in literature and are predominantly observed in elderly or in immunocompromised patients. The clinical manifestations of skin infections include granulomatous lesions, necrotizing fasciitis, nodules, cellulitis, ulcers, dermal abscesses. Infections caused by S. marcescens may be difficult to treat because of resistance to a variety of antibiotics, including ampicillin and first and second generation cephalosporins. Aminoglycosides have good activity against S. marcescens, but resistant strains have also been described. We report a very intriguing case of S. marcescens infection, in an immunocompetent 18-year-old man, causing multiple rounded ulcers of varying sizes, along with few pustular lesions that both clinically and histopathologically mimic a pyoderma gangrenosum (PG). This is a non infectious neutrophilic skin disorder, characterized by painful and rapidly progressing skin ulceration. According to our experience, we would strongly recommend to perform cultures of multiple skin ulcers resembling PG, even in young healthy patients, to ensure correct diagnosis and treatment, since resistant to conventional antibiotics bacteria such as S. marcescens may be the cause of these lesions, like in the case here reported.

A favourable response to surgical intervention and hyperbaric oxygen therapy in pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterised with ulcerations. Inflammatory bowel diseases (ulcerative colitis and Crohn's disease) and haematologic diseases (leukaemia, preleukaemia and monoclonal gammopathy) have been reported in about 40-50% of PG patients in whom the treatment of the underlying disease is important for the improvement of the lesions. We herein report a colorectal adenocarcinoma patient with PG, who responded partially to topical treatments and systemic immunosuppressants and healed completely with the aid of surgical wound repair and hyperbaric oxygen therapy.

Autoinflammatory skin disorders in inflammatory bowel diseases, pyoderma gangrenosum and Sweet's syndrome: a comprehensive review and disease classification criteria.

Pyoderma gangrenosum (PG) and Sweet's syndrome (SS) are skin diseases usually presenting with recurrent ulcers and erythematous plaques, respectively. The accumulation of neutrophils in the skin, characteristic of these conditions, led to coin the term of neutrophilic dermatoses to define them. Recently, neutrophilic dermatoses have been included in the group of autoinflammatory diseases, which classically comprises genetically determined forms due to mutations of genes regulating the innate immune response. Both PG and SS are frequently associated with inflammatory bowel diseases (IBDs); however, IBD patients develop PG in 1-3 % of cases, whereas SS is rarer. Clinically, PG presents with deep erythematous-to-violaceous painful ulcers with well-defined borders; bullous, pustular, and vegetative variants can also occur. SS is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions, and a diffuse neutrophilic dermal infiltrate. It is also known as acute febrile neutrophilic dermatosis. Treatment of PG involves a combination of wound care, topical medications, antibiotics for secondary infections, and treatment of the underlying IBD. Topical therapies include corticosteroids and the calcineurin inhibitor tacrolimus. The most frequently used systemic medications are corticosteroids and cyclosporine, in monotherapy or in combination. Dapsone, azathioprine, cyclophosphamide, methotrexate, intravenous immunoglobulins, mycophenolate mofetil, and plasmapheresis are considered second-line agents. Hyperbaric oxygen, as supportive therapy, can be added. Anti-TNF-α agents such as etanercept, infliximab, and adalimumab are used in refractory cases. SS is usually responsive to oral corticosteroids, and the above-mentioned immunosuppressants should be considered in resistant or highly relapsing cases.

Actualización en tratamiento de pioderma gangrenoso: revisión de la literatura / Update on the treatment of pyoderma gangrenosum: literature review

Pioderma gangrenoso es una patología inflamatoria crónica, poco frecuente, de probable etiología autoinmune. Se asocia en 50 por ciento de los casos a múltiples enfermedades sistémicas. En su forma clásica, se presenta como una lesión ulcerativa, dolorosa, habitualmente en extremidades inferiores. El diagnóstico es clínico. La histología es inespecífica, pero permite descartar otras patologías como infecciones o neoplasias. No existe hasta el momento un único tratamiento efectivo para esta enfermedad. Los corticoides sistémicos en dosis continua o en pulsos, son efectivos en la mayoría de los casos. Se reserva el uso de inmunosupresores para los casos refractarios a tratamiento. Las nuevas terapias biológicas han mostrado buenos resultados, especialmente en pacientes con enfermedad inflamatoria intestinal asociada.

Pyoderma gangrenosum is a chronic inflammatory disease of probable autoimmune etiology. It is associated in 50 percent of cases to multiple systemic diseases. It usually presents as a painful ulcerative lesion. The legs are most commonly affected. The diagnosis is based on the clinical presentation. The objective of biopsy is to rule out other causes of ulceration, such as infections or malignancies. There is no specific and uniformly effective therapy for this disease. Systemic corticosteroids, administered continuously or in pulses, have been an effective treatment in most cases. We reserve the use of immunosuppressant for cases refractory to treatment. New biological therapies have shown good results, especially in patients with inflammatory bowel disease.

Pyoderma gangrenosum: a report of a rare complication after knee arthroplasty requiring muscle flap cover supplemented by negative pressure therapy and hyperbaric oxygen.

Pyoderma gangrenosum (PG) is rare ulcerating skin condition easily confused with wound infection following surgery. We report a complicated case of PG following knee arthroplasty where delayed diagnosis and repeated debridements lead to significant tissue loss. Successful reconstruction was achieved with a muscle flap, but subsequent reactivation of PG and superadded infection placed both the reconstruction and patient's life at risk. Prolonged combined use of negative pressure therapy (NPT), immunosuppression and hyperbaric oxygen (HBO) was successfully used to reduce the wound size, enhance wound granulation, promote re-epithelialisation, and provide pain relief. There is little or no published literature on these treatment modalities for the management of PG, with only one reported case using both NPT and HBO for PG (not following knee arthroplasty). More studies are necessary to determine the role of both modalities in the management of pathergy in large and complex wounds and the rare nature of this complication following knee arthroplasty explains the lack of evidence-based guidance. In conclusion, we suggest a surgical algorithm. This is the first report of PG following knee arthroplasty with the use of both NPT and HBO in order to achieve soft tissue coverage.

[Pyoderma gangrenosum: report of three cases in plastic surgery]. / Pioderma gangrenosa en cirugía plástica: comunicación de tres casos.

Pyoderma Gangrenosum is a rare disease whose etiology is probably autoimmune. We report two males aged 48 and 49 years and one female aged 54 years, with lesions in the right thigh, right leg and in the borders of a surgical incision in the chest. This article gives a brief description of the lesions, tips for diagnosis and help for the initial management and treatment.

Pioderma gangrenosa en cirugía plástica: Comunicación de tres casos / Pyoderma gangrenosum: Report of three cases in plastic surgery

Pyoderma Gangrenosum is a rare disease whose etiology is probably autoimmune. We report two males aged 48 and 49 years and one female aged 54 years, with lesions in the right thigh, right leg and in the borders of a surgical incision in the chest. This article gives a brief description of the lesions, tips for diagnosis and help for the initial management and treatment.

Treatment of pyoderma gangrenosum with oral Tripterygium wilfordii multiglycoside.

Two patients with refractory pyoderma gangrenosum (PG) were treated with oral Tripterygium wilfordii multiglycoside (TWG). TWG is a Chinese medicine extracted from a medicinal herb, Tripterygium wilfordii Hook F, and has potent anti-inflammatory and immunosuppressive effects. The effect of TWG on PG was demonstrated by clinical findings. Improvement of the lesions occurred within two weeks, and the ulcers healed about a month. Mild side effects such as gastrointestinal disturbances were observed in both patients. These side effects were patient-acceptable, and there was no need to stop the treatment. Transient elevation of serum ALT was observed in one patient; the serum ALT returned completely to normal after the discontinuation of TWG. These results suggest that TWG may be an effective alternative for refractory PG and that careful monitoring of liver function during TWG treatment is necessary.

Pioderma gangrenoso: revisión de 29 casos clínicos / Pyoderma gangrenosum: a review of 29 patients

Una revisión de 29 pacientes con diagnóstico de Pioderma Gangrenoso es analizado en cuanto a la clínica, forma de presentación, asociaciones, respuesta al tratamiento y recaidas, entre otros; comparándolo finalmente con lo reportado en la literatura (AU)

Pyoderma gangrenosum of the orbit.

Pyoderma gangrenosum is an uncommon chronic ulcerative condition, the aetiology of which is poorly understood. Ophthalmic involvement is rare. The case presented in this paper involves destruction of the orbital contents with subsequent perforation of the eye despite conventional therapy. Evisceration was performed with the patient being subjected to hyperbaric oxygen therapy pre- and post-operatively, with resultant cessation of the disease process.