Research advances on the pathogenesis and diagnosis of pyoderma gangrenosum / 中华烧伤杂志

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis, closely associated with the immune system. Its pathogenesis is currently not clear. The lack of specificity in the clinical manifestations and histopathological changes of PG leads to a long clinical diagnosis cycle and even misdiagnosis, which is easy to delay treatment or promote the deterioration of ulcer wound. The diagnosis of this disease is still very difficult, which poses a great challenge to wound repair practitioners. This article reviews the research advances on the pathophysiology, clinical features, and diagnosis of PG in recent years, with the aim of providing reference for relevant clinical practitioners.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Pioderma gangrenoso en mamoplastia posbariátrica / Pyoderma gangrenosum in postbariatric mammoplasty

El pioderma gangrenoso es una patología infrecuente, de origen desconocido, caracterizada por la presencia de úlceras cutáneas de carácter necrótico, de aparición recurrente y rápida progresión. Puede manifestarse en cualquier área anatómica a partir de procesos quirúrgicos o traumáticos, que actúan como disparadores de la reacción autoinmune localizada. En pacientes con cirugía reciente, los signos y síntomas de esta patología se asemejan a una infección posoperatoria. El tratamiento se basa en el uso de corticosteroides e inmunosupresores, containdicando la conducta quirúrgica dado que la misma puede exacerbar la enfermedad. Presentamos un caso de pioderma gangrenoso en mastoplastía posbariátrica con inclusión de prótesis.

Pyoderma gangrenosum is a rare pathology of unknown origin, characterized by the presence of necrotic skin ulcers of recurrent appearance and rapid progression. It can emerge in any anatomic area, after surgeries or traumatic procedures which act as triggers of the localized immune reaction. In patients who have undergone recent surgeries, the signs and symptoms from this pathology resemble a post-operative infection. The treatment consist of corticosteroids and/or immunosuppressants. Surgical procedure is contraindicated since it can exacerbate the disease. We present a case of pyoderma gangrenosum in mastopexy with breast implant insertion post bariatric surgery.

PsAPASH: a rare and recent autoinflammatory syndrome associated with hidradenitis suppurativa

Abstract Hidradenitis suppurativa is a chronic inflammatory skin disease, which affects 1% of the population, being more common in young, obese and smokers, and mainly affects armpits and groin, with formation of pustules, nodules, abscesses, scars and fistulas. Recently, its association with other autoimmune diseases such as psoriasis, psoriatic arthritis, pyoderma gangrenosum, pyogenic arthritis and ulcerative colitis have been reported. These associated forms are usually resistant to standard treatment, with immunobiologicals as promising therapy. The case of a rare form of association is reported, with only one case previously described in the literature: psoriasis arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa.

Pyoderma gangrenosum: a review with special emphasis on Latin America literature

Abstract Pyoderma gangrenosum is a neutrophilic dermatosis characterized by chronic ulcers due to an abnormal immune response. Despite the existence of diagnostic criteria, there is no gold standard for diagnosis or treatment. In Latin America, recognizing and treating pyoderma gangrenosum is even more challenging since skin and soft tissue bacterial and non-bacterial infections are common mimickers. Therefore, this review aims to characterize reported cases of pyoderma gangrenosum in this region in order to assist in the assessment and management of this condition. Brazil, Mexico, Argentina, and Chile are the countries in Latin America that have reported the largest cohort of patients with this disease. The most frequent clinical presentation is the ulcerative form and the most frequently associated conditions are inflammatory bowel diseases, inflammatory arthropaties, and hematologic malignancies. The most common treatment modalities include systemic corticosteroids and cyclosporine. Other reported treatments are methotrexate, dapsone, and cyclophosphamide. Finally, the use of biological therapy is still limited in this region.

Extensive pyoderma gangrenosum-like lesions revealing a case of hyperzincemia and hypercalprotectinemia: when to suspect it?

Abstract Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases.

PLECT or PPLECT? Granulomatous pyoderma gangrenosum in the differential diagnosis of the verrucous syndrome

Abstract: Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis, with variable clinical features, nonspecific histopathology and multifactorial pathogenesis, posing a challenging diagnosis for the dermatologist. Pyoderma gangrenosum is a diagnosis of exclusion and should be included in the differential diagnoses of the verrucous syndrome. We report a granulomatous variant affecting the face.

Bullous pyoderma gangrenosum as a predictor of hematological malignancies

Abstract: Pyoderma gangrenosum may precede, coexist or follow diverse systemic diseases. The bullous variety is usually associated with hematologic disorders. From 31 patients with pyoderma gangrenosum diagnosed in our hospital during 10 years, only 2 presented with the bullous type. One patient had previous diagnosis of chronic myeloid leukemia. Both had fast-progressive, painful lesions, and both showed an excellent response to systemic corticosteroids. In less than 3 months the lesions recurred simultaneously with the progression of chronic myeloid leukemia in one patient and with the initial diagnosis of acute myeloid leukemia in the other one. They died in less than four weeks. These findings emphasize the importance of its timely diagnosis. Cutaneous lesions could be the first predictor of an underlying disease.

Exuberant pyoderma gangrenosum in a patient with autoimmune hepatitis

Abstract: Pyoderma gangrenosum is a rare neutrophilic dermatosis, which usually presents as ulcers with erythematous-violaceous undermined edges and a rough base with purulent or sanguinous exudate. It can be primary or associated with an underlying disease. However, rare cases of its association with autoimmune hepatitis have been described in the literature. Diagnosis is based on a characteristic clinical picture and ruling out other causes of ulcers. This paper aims to discuss the management of corticosteroid therapy and the importance of local treatment. We report a case with torpid evolution, presented with multiple and deep ulcers in a young patient with autoimmune hepatitis, causing pain and significant disability. We observed complete healing of lesions after two months of successful treatment.

Pyoderma gangrenosum associated with left iliac vein compression syndrome: presentation of difficult diagnosis

Abstract: Pyoderma gangrenosum is a rare neutrophilic dermatosis of unknown etiology, of which the most frequent clinical manifestations are ulcers. The diagnosis difficulty is, among other things, to rule out other causes of ulcers, since it is considered a diagnosis of exclusion. Skin ulcerations may also occur in the iliac vein compression syndrome, which, like pyoderma gangrenosum, mainly affects young women. Because they have such similar characteristics, the presence of vascular disease may hinder the diagnosis of concurrent pyoderma gangrenosum. Because of the clinical relevance of ulcerated lesions and scars, the early diagnosis and treatment of this condition is considered extremely important. We report a case in which the two diseases were associated, hampering the diagnosis of pyoderma gangrenosum.

Pioderma gangrenoso em abdominoplastia: relato de caso / Pyoderma gangrenosum in abdominoplasty: a case report

Pioderma gangrenoso é uma doença caracterizada por úlceras dolorosas, com bordos imprecisos, de diversos tamanhos e profundidade. O diagnóstico é eminentemente clínico, sendo muitas vezes de exclusão. O tratamento não é específico. O desbridamento cirúrgico não é indicado devido a seu potencial de patergia. Neste trabalho, relatamos um caso de pioderma gangrenoso em uma paciente submetida à abdominoplastia.

Pyoderma gangrenosum is a disease characterized by painful ulcers, with imprecise borders of various sizes and depth. The diagnosis is essentially clinical and often exclusionary. The treatment is not specific. Surgical debridement is not indicated because of its potential pathergy. We report a case of pyoderma gangrenosum in a patient who underwent abdominoplasty.

Pyoderma Gangrenosum: A Case Series.

Pyoderma gangrenosum is a rare, ulcerative, non-infectious neutrophilic dermatosis, commonly associated with underlying systemic disease. The features of pyoderma gangrenosum are not specific histopathologically and for this reason the diagnosis is based on clinical feature. The systemic administration of corticosteroids is the mainstay of treatment. We present five cases of pyoderma gangrenosum of age group ranging from 2 to 75 years. One of these patients was HIV-infected, and one was having discoid lupus erythematosus. The lesions were present on thighs in 3 cases, over back in one and breast in one. Biopsy showed central necrosis with multiple neutrophilic abscesses in epidermis. The dermis showed dense neutrophilic infiltration in both superficial and deep dermis. The lesions responded well to oral corticosteroids except in the case of HIV-infected patient in which minocycline showed considerable improvement.

Pioderma gangrenoso após quadrantectomia de mama com radioterapia intraoperatória: relato de caso / Pyoderma gangrenosum after quadrantectomy with intraoperative radiotherapy: a case report

Pioderma gangrenoso é uma doença inflamatória imunomediada e rara da pele, de diagnóstico extremamente desafiador. A evolução clínica é a base para o diagnóstico, cursando com lesões pustulosas superficiais, halo eritematoso doloroso, rápida progressão para ulcerações dolorosas e estéreis, sem resposta a antibióticos ou a novas intervenções cirúrgicas e, finalmente, com pronta melhora com uso de imunossupressores. O atraso no diagnóstico pode acarretar numerosas internações e terapias prolongadas, sendo que seu reconhecimento precoce, por outro lado, evita a progressão dessas ulcerações e sua morbidade. Relatou-se um caso de pioderma gangrenoso que evoluiu após cirurgia associada à radioterapia intraoperatória no tratamento conservador do câncer de mama, fazendo-se uma revisão de casos relatados na literatura e suas possibilidades terapêuticas. Questiona-se, também, se a radioterapia intraoperatória estaria relacionada com algum estímulo imunomediado, o que poderia ter facilitado o desencadeamento do quadro.

Pyoderma gangrenosum is an immune-mediated inflammatory and rare skin disease with an extremely challenging diagnosis. The clinical evolution of the disease is the basis for the diagnosis that involves pustular superficial lesions, painful erythematous halo, rapid progression to painful and sterile ulcerations, unresponsiveness to antibiotics or new surgical interventions, and finally, ready improvement with the use of immunosuppressive drugs. Delayed diagnosis may cause numerous hospitalizations and prolonged therapy, whereas early recognition can prevent the progression of the ulcerations and their morbidities. We report a case of pyoderma gangrenosum that evolved after surgery and was associated with intraoperative radiotherapy for the conservative treatment of breast cancer. In addition, we reviewed reported cases in the literature and therapeutic options. It is conjectured that intraoperative radiotherapy might be related to some immune-mediated stimuli that could trigger the clinical condition.

Pioderma gangrenoso associado à artrite reumatoide: descrição de caso / Pyoderma gangrenosum associated with rheumatoid arthritis: a case report

O pioderma gangrenoso é uma dermatose inflamatória crônica, que se associa a doenças sistêmicas como a artrite reumatoide. É mais comum em adultos e pode se apresentar com quatro formas clínicas, todas levando à ulceração da pele acometida. Seu diagnóstico é clínico e de exclusão. O tratamento deve ser realizado com cuidados locais e terapia sistêmica.

Pyoderma gangrenosum is a chronic inflammatory dermatosis, which is associated with non-infectious systemic diseases such as rheumatoid arthritis and inflammatory bowel disease. It is more common in adults and may present with four distinct clinical forms, all leading to ulceration of the skin affected. Its diagnosis is clinical and demands exclusion of other causes. Treatment should be performed with local care and systemic therapy.

Pioderma gangrenoso / Pyoderma gangrenosum

El pioderma gangrenoso (PG) es una enfermedad de etiología desconocida, que se incluye en el grupo de las dermatosis neutrofílicas. Se reconocen cuatro variantes clínicas: ulcerativa,ampollar, pustulosa y vegetante. Puede presentarse en asociación con una enfermedad sistémica en aproximadamente el 50% de los casos. Para el diagnóstico es importantela exclusión de otras patologías, ya que no hay hallazgos específicos de laboratorio ni histopatológicos. Su manejo requiere tratamiento local y con frecuencia sistémico. Se presentan dos casos de PG ulcerosa...

Pyoderma gangrenosum with renal and splenic impairment - case report / Manifestacao esplenica e renal do pioderma gangrenoso - relato de caso

Pyoderma gangrenosum is an uncommon and recurrent neutrophilic dermatosis of unknown cause. The lesions usually start as tender sterile papulopustules or erythematous nodules that undergo necrosis followed by ulceration. The lower limbs are most commonly affected and around half of the cases are associated with systemic disorders. Although rare, cases of pyoderma gangrenosum with extramucocutaneous sterile neutrophilic infiltrate have been reported, with the lungs being the most commonly affected organ. We report a case of pyoderma gangrenosum with splenic and renal impairment. Pyoderma gangrenosum should be considered a multisystemic disease with classic cutaneous manifestations and potential involvement of internal organs.

Pioderma gangrenoso é uma dermatose neutrofílica incomum, recorrente e de etiologia desconhecida. As lesões geralmente se iniciam como papulopústulas estéreis ou nódulos eritematosos que sofrem necrose seguida de ulceração. Os membros inferiores são mais comumente afetados e quase a metade dos casos está associada a doenças sistêmicas. Apesar de raros, casos de pioderma gangrenoso com infiltrado neutrofílico estéril extramucocutâneo foram relatados e os pulmões são os órgãos mais comumente acometidos. Nós relatamos um caso de manifestação esplênica e renal do pioderma gangrenoso. Pioderma gangrenoso deve ser considerado doença multissistêmica com manifestações cutâneas clássicas e potencial envolvimento de órgãos internos.

Pyoderma gangrenosum in a newborn - case report / Pioderma gangrenoso em recem - nascido - relato de caso

Pyoderma gangrenosum is a rare, inflammatory, chronic and recurrent disease of unknown etiology, characterized by noninfectious, necrotizing and painful cutaneous ulcers. Usually it affects adults aged between 25 and 54 years old and rarely children (less than 4%), in which it mainly affects the head, face, buttocks, genital and perianal region. The disease presents a quick response to systemic corticosteroids. We report a case of a newborn with hemorrhagic and necrotic ulcers, distributed in the abdomen, buttocks and genital region with rapid and effective response to oral prednisone.

O Pioderma gangrenoso é uma doença inflamatória rara, crônica e recorrente, de etiopatogenia desconhecida, caracterizada por uma ulceração não-infecciosa, necrotizante e dolorosa da pele. Geralmente, afeta adultos na faixa de 25 a 54 anos e raramente crianças (menos de 4%), nestes acomete preferencialmente cabeça e face, nádegas, região perianal e genital. Apresenta rápida resposta à corticoterapia sistêmica. Relata-se um caso de recém-nascida com lesões ulceradas de pioderma gangrenoso de fundo hemorrágico e necrótico distribuídas em abdômen, região genital e glútea com resposta rápida e efetiva à prednisona oral.

Pyoderma gangrenosum: skin grafting and hyperbaric oxygen as adjuvants in the treatment of a deep and extensive ulcer / Pioderma gangrenoso: enxerto de pele e oxigenoterapia hiperbarica como adjuvantes no tratamento de ulcera extensa e profunda

Pyoderma gangrenosum is a rare dermatosis of unknown etiology and variable clinical presentation. The disease is challenging for the medical staff, from the frequent diagnostic difficulties to the lack of scientific evidence with a good level to support the management of extensive and refractory cases. Our patient is a 50 year-old man with an extensive and deep ulcer on the left leg, which exemplifies the therapeutic difficulties inherent to the disease and who, fortunately, has progressed with excellent result after association of hyperbaric oxygen therapy and skin grafting to the immunosuppression therapy initially proposed.

Pioderma gangrenoso é dermatose rara de etiologia não completamente compreendida e apresentação clínica variável. A doença apresenta muitos desafios à equipe médica, desde a freqüente dificuldade diagnóstica até a inexistência de evidências científicas com bom nível para condução de casos extensos e refratários. Apresentamos o caso de um paciente masculino, 50 anos, com úlcera extensa e profunda em perna esquerda, que exemplifica bem a dificuldade terapêutica e que, felizmente, evoluiu com excelente resultado após associação de oxigenoterapia hiperbárica e enxertia de pele ao tratamento imunossupressor inicialmente proposto.

Pioderma Gangrenoso: um Artigo de Revisão / Pyoderma Gangrenosum: a Review Article

O pioderma gangrenoso (PG) é uma dermatose crônica com características peculiares e de etiologia desconhecida, muitas vezes de difícil diagnóstico. Manifesta-se através de lesões cutâneas ulceradas e dolorosas com evolução rápida e progressiva, mais comumente em membros inferiores. As ulcerações podem surgir espontaneamente ou depois de variados tipos de trauma. O período entre o início das lesões e o diagnóstico correto costuma ser prolongado. Não existe nenhum tratamento padronizado ou algoritmo simples para a escolha da terapia. Neste artigo, os autores fazem uma ampla revisão da literatura atual acerca da fisiopatologia, do diagnóstico e do tratamento desta patologia através de análise sistemática das referências bibliográficas atuais nas bases de dados PubMed, Scielo, Medline e Lilacs.

Pyoderma gangrenosum (PG) is a chronic dermatosis with peculiar characteristics and unknown etiology, often of difficult diagnosis. It manifests as painful skin ulcers with rapid and progressive evolution, most commonly in the lower limbs. Ulcerations can occur spontaneously or after various types of trauma. The period between the onset of the lesions and the correct diagnosis is often prolonged. There is no standard treatment or simple algorithm for the choice of therapy. In this article, we present a comprehensive review of the current literature on the pathophysiology, diagnosis, and treatment of this pathology through systematic analysis of the current references in the databases PubMed, Scielo, Medline and Lilacs.