Current situation and the latest progress in the treatment of pyoderma gangrenosum / 中华烧伤杂志

Pyoderma gangrenosum (PG) is a rare chronic inflammatory non-infectious skin dermatosis, and there is no clear treatment guideline for this disease at home and abroad. There are a variety of clinical treatment methods for PG, including local therapy and systemic application of glucocorticoids, immunosuppressants, intravenous immuno- globulin, and biologics. Glucocorticoids are the first-line drugs commonly used in clinical practice, and immunosuppressants can be used alone or in combination with glucocorticoids. In recent years, more and more evidence has shown that biologics are a new trend in the treatment of PG, mainly including tumor necrosis factor α inhibitors, interleukin-1 (IL-1) inhibitors, IL-12/23 inhibitors, IL-17 inhibitors, rituximab, and small molecular inhibitors. This article summarizes the current status and latest progress in the treatment of PG, hoping to provide clinicians with ideas for the treatment of PG.

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)

Pyoderma gangrenosum associated to the use of cocaine/levamisole. Series of three cases and literature review

Abstract Pyoderma gangrenosum associated to the use of cocaine/levamisole is a rare condition associated to their consumption. Cocaine use is frequent in Colombia, and the substance is contaminated with levamisole, an anthelmintic that increases the psychotropic effects and enhances its side effects. We present three clinical cases of patients with ulcerated lesions, in which the diagnosis was pyoderma gangrenosum secondary to the use of cocaine contaminated with levamisole. This called the attention of the health staff to investigate the abuse of substances in gangrenous pyoderma and also evidence that the interruption of consumption was the basis of management.

Tratamiento exitoso del pioderma grangrenoso con metotrexate en paciente con enfermedad de Crohn / Successful treatment of gangrenous pyoderma with methotrexate in a patient with Crohn's disease

El pioderma gangrenoso (PG), es una dermatosis neutrofílica, cutáneo-ulcerativa, no infecciosa, que ocurre hasta en un 2% de los pacientes con enfermedad de Crohn (EC). Su aparición suele ser independiente del curso clínico de la EC. Las terapias más utilizadas han sido los corticoides sistémicos, la ciclosporina y la terapia biológica, los cuales han mostrado buenos resultados. El Metotrexate (MTX) es un fármaco antimetabolito con actividad antiinflamatoriaque se caracteriza por una dosificación semanal, un inicio de acción más rápida, con buen perfil de seguridad relacionado con neoplasias malignas y sobretodo un menor costo. Posee eficacia en la inducción de la remisión en pacientes con EC, sin embargo su eficacia en el manejo de manifestaciones extraintestinales como el PG es incierta. Presentamos el caso de un varón joven que acude a urgencias por diarrea crónica, pérdida de peso, y fiebre acompañado de un nódulo doloroso, eritematoso, situado a nivel pretibial compatible con PG en el contexto de EC. Fue manejado inicialmente con corticoides sistémicos sin mejoría. De tal forma, se inició terapia concomitante con MTX con respuesta clínica y analítica favorable. En conclusión, la terapia concomitante de cortiocoides sistémicos y MTX en pacientes con EC con PG podría suponer un tratamiento alternativo al no contar con disponibilidad de ciclosporina o terapia biológica en nuestro medio.

Pyoderma gangrenosum (PG) is a neutrophilic, cutaneous-ulcerative, non-infectious dermatosis that occurs in up to 2% of patients with Crohn's disease (CD). Its appearance is usually independent of the clinical course of CD. The most used therapies have been systemic corticosteroids, cyclosporine and biological therapy, which have shown good results. Methotrexate (MTX) is an antimetabolite drug with anti-inflammatory activity characterized by a weekly dosage, a faster onset of action, with a good safety profile related to malignant neoplasms and, above all, a lower cost. It is effective in the induction of remission in patients with CD, however its efficacy in the management of extraintestinal manifestations such as PG is uncertain. We present the case of a young man who comes to the emergency room with an history of chronic diarrhea, weight loss, and fever accompanied by a painful, erythematous nodule, located at the lower extremity compatible with PG in the context of CD. It was initially managed with systemic corticosteroids without improvement. Concomitant therapy with MTX was started with a favorable clinical outcome. In conclusion, the concomitant therapy of systemic corticosteroids and MTX in patients with CD with PG could be an alternative treatment in cases where cyclosporine or biological therapy is not available.

Pioderma gangrenoso em pós-operatório de mamoplastia redutora: relato de caso / Pyoderma gangrenosum following reduction mammoplasty: a case report

O pioderma gangrenoso (PG) é uma dermatose neutrofílica inflamatória, de etiologia desconhecida. O PG é idiopático em 25-50% dos casos. Em aproximadamente 50% dos casos tem sido descrita a associação com doenças sistêmicas, tais como: doença de Crohn, gamopatias monoclonais, artrites soropositivas, colagenoses, doença de Behcet, granulomatose de Wegener, doenças mieloproliferativas e infecciosas, principalmente hepatites e Aids. Clinicamente, apresenta quatro variantes: ulcerada, bolhosa, vegetante e pustulosa. A forma mais frequente é a ulcerativa, que se inicia com pápula ou nódulo e evolui rapidamente para lesões ulceradas e dolorosas. Em até 25% dos casos de PG, o surgimento de novas lesões pode ser desencadeado por traumas, tais como picadas de insetos, injeções intravenosas e biópsia - fenômeno conhecido por patergia. Nesse trabalho, é apresentado um caso de PG extenso das mamas em pós-operatório de mamoplastia redutora, de difícil diagnóstico; iniciado na evolução pós-operatória em outro serviço. Devido à quebra da relação médico-paciente, vem procurar nosso serviço. Apresentou ótima resposta ao tratamento com corticoterapia (corticoterapia intra e perilesionais com triancinolona) no ato do desbridamento, e introdução de corticoterapia via oral (prednisona) em esquema escalonado de desmame.

Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis of unknown etiology and is idiopathic in 25%- 50% cases. In approximately 50% of PG cases, an association with systemic diseases, such as Crohn's disease, monoclonal gammopathies, seropositive arthritis, collagenosis, Behcet's disease, Wegener's granulomatosis, and myeloproliferative and infectious diseases (mainly hepatitis and AIDS), has been described. Clinically, PG presents four variants: ulcerated, bullous, vegetative, and pustular. The most frequent form is ulcerative, which begins as a papule or nodule and evolves rapidly into ulcerated and painful lesions. In approximately 25% of PG cases, the onset of new lesions can be triggered by traumas such as insect bites, intravenous injections, and biopsy, a phenomenon known as pathergy. Here, we present a case of extensive PG of the breasts following reductive mammoplasty surgery. It was a difficult case to diagnose and was initiated in the postoperative period of another service. Due to the breakdown of the doctor-patient relationship, the patient approached us for assistance. The patient showed an excellent response to corticotherapy (intra and perilesional corticotherapy with triamcinolone) during debridement and oral steroid (prednisone) therapy in the weaning phase.

Oligoartritis, paniculitis y pioderma gangrenoso como manifestaciones de un Linfoma no Hodgkin / Oligoarthritis, panniculitis and pyoderma gangrenosum associated with non-Hodgkin lymphoma: report of one case

An assortment of clinical and laboratory abnormalities may occur as paraneoplastic syndromes in lymphomas. Rheumatological and dermatological manifestations such as paraneoplastic arthritis and pyoderma gangrenosum must be underscored. We report a 28 years old woman who developed pyoderma gangrenosum and two years later presented with arthritis of knees and ankles associated with panniculitis interpreted as erythema induratum that was pathologically confirmed. She developed a reactivation of pyoderma gangrenosum, that was refractory to treatment. Complementary studies showed a pulmonary nodule and a right paravertebral mass with involvement of the psoas muscle. Biopsies of both masses and a new pathological skin study demonstrated a large B-cell non-Hodgkin's lymphoma.

Pioderma gangrenoso vulvar: revisión de la literatura a propósito de un caso causado por rituximab / Vulvar pyoderma gangrenosum: review of the literature on a case caused by rituximab

RESUMEN El Pioderma Gangrenoso (PG) es una enfermedad inflamatoria necrotizante crónica, que pertenece al espectro de las dermatosis neutrofílicas. Histológicamente se caracteriza por mostrar un infiltrado inflamatorio denso de neutrófilos de origen no infeccioso. El PG suele asociarse a enfermedades sistémicas como la enfermedad inflamatoria intestinal, la artritis reumatoide o diversas enfermedades hematológicas. Presenta fenómeno de patergia y suele responder satisfactoriamente a tratamientos inmunosupresores. Su etiología no está bien definida. En la literatura se han publicado 15 casos de pioderma gangrenoso vulvar asociado al uso de rituximab. Nosotros presentamos un nuevo caso, que tuvo lugar en una mujer de 37 años en tratamiento de mantenimiento con rituximab por un linfoma no Hodgkin folicular. El rituximab (MabThera®) es un anticuerpo que reconoce la molécula CD20, que es una proteína no glucosilada que se expresa en la superficie de los linfocitos B. Este fármaco se ha utilizado para el tratamiento de diferentes enfermedades reumatológicas en los últimos años.

ABSTRACT Pyoderma Gangrenosum is a chronic necrotizing inflammatory disease that belongs to the spectrum of Neutrophilic Dermatoses. Histologically, it is characterized by a dense inflammatory infiltrate of non-infectious neutrophils. Etiology is not yet well defined. It is usually associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis or hematological diseases. It presents pathergy phenomenon and usually respond satisfactorily to immunosuppressive treatments. There have been published only 15 cases of vulvar pyoderma gangrenosum associated with the use of rituximab. We present a new case, which occurred in a 37-year-old woman on maintenance treatment with rituximab for a follicular non-Hodgkin's lymphoma. Rituximab (MabThera®) is an antibody that recognizes the CD20 molecule, which is a non-glycosylated protein that is expressed on the surface of B lymphocytes. This drug has been used for the treatment of different rheumatic diseases in recent years.

Pioderma gangrenoso ulceroso asociado a artritis reumatoide: reporte de caso / Ulcerative pyoderma gangrenosum associated with rheumatoid arthritis: a case report

ResumenEl pioderma gangrenoso corresponde a una dermatosis crónica inflamatoria rara, cuya etiología y patogénesis es aún incierta, aunque factores inmunológicos y disfunción neutrofílica parecen desempeñar un papel importante. Se presenta en la mayoría de los casos asociada a enfermedad sistémica no infecciosa, como artritis reumatoide, malignidad hematológica y enfermedad inflamatoria intestinal (EII). Es más común en mujeres entre la tercera y quinta década de la vida y puede presentarse como cuatro variantes clínicas distintas que conllevan a ulceración de la piel, con topografía predominante a nivel de extremidades inferiores. Su diagnóstico es meramente clínico y de exclusión y el tratamiento a menudo amerita medidas locales y sistémicas.Se describe aquí el caso clínico de una paciente femenina de 38 años con pioderma gangrenoso variante ulcerativa, asociado a artritis reumatoide, quien presentó excelente evolución clínica posterior a instaurado el tratamiento esteroideo sistémico.

AbstractPyoderma gangrenosum is a rare chronic inflammatory dermatosis of unknown etiology and pathogenesis, although immunological factors and neutrophil dysfunction are believed to play an important role. In most cases it is associated with non-infectious systemic diseases such as rheumatoid arthritis, hematologic malignancies and inflammatory bowel disease. It is more common in women with a peak incidence between the third and fifth decades of life and it may present in four distinct clinical forms, all leading to ulceration of the skin, with involvement predominantly of the lower limbs. The diagnosis is based on the clinical manifestations and exclusion of other entities. Frequently systemic and local therapy is required.The clinical case of a 38 year old woman with ulcerative pyoderma gangrenosum associated with rheumatoid arthritis who had an excellent response to treatment with systemic steroids is described.

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: 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 gigante de curso fulminante asociado a enfermedad inflamatoria intestinal / Giant pyoderma gangrenosum associated with an inflammatory bowel disease

Background: Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin. Case report: We report a 23 years old male subjected to a total colectomy and ileostomy due to a toxic megacolon associated with an inflammatory bowel disease. Ten months later, he suffered an infected abdominal wall dehiscence after a surgical intervention for a bowel obstruction. The patient presented confluent ulcerated skin lesions surrounding the ileostomy and drainage sites, associated with severe malaise and without response to antimicrobial treatment. Presuming the presence of a giant and fulminant pyoderma gangrenosum associated with Crohn's disease, the patient was treated with adrenal steroids and immunosuppressive agents. One month later an enterocutaneous fistula appeared. Thereafter the patient experienced a slow but progressive recovery with a complete healing of the skin lesion and closure of enterocutaneous fistula after four years of immunosuppressive and anti-tumor necrosis factor treatment. The basis of the treatment of this patient was the use of immunosuppressive agents.

Introducción: El pioderma gangrenoso (PG) es una enfermedad cutánea crónica, probablemente de etiología autoinmunitaria, que se manifiesta en la mayoría de los casos como una úlcera dolorosa. Caso clínico: Se presenta el caso de un paciente de 23 años sometido a una colectomía total e ileostomía por megacolon tóxico asociado a una enfermedad inflamatoria intestinal. A los 10 meses, tras una intervención por obstrucción intestinal presenta una infección con dehiscencia de la pared abdominal y grandes zonas ulceradas confluentes peri-ileostómicas y en el sitio del drenaje, en el contexto de un grave compromiso del estado general que no responde a múltiples esquemas antibióticos. Por la forma clínica de presentación, el diagnóstico de esta condición fue elusivo. Con la colaboración de un equipo multidisciplinario y el diagnóstico presuntivo de pioderma gangrenoso (PG) gigante de curso fulminante asociado a una enfermedad de Crohn, se inicia corticoterapia asociado a inmunosupresores, que cambia el curso ominoso de la enfermedad, logrando una recuperación lenta pero progresiva del estado general y una cicatrización completa de la pared abdominal luego de 4 años de tratamiento y el cierre de una fístula entero-cutánea que surge en la evolución de la ulceración central. Conclusiones: Los pilares del tratamiento en este inusual caso de PG fueron la corticoterapia asociada a inmunosupresores en la fase aguda de la enfermedad, seguida de un agente biológico inhibidor del factor de necrosis tumoral (infliximab) en la fase de mantención.

Pioderma gangrenoso en mamoplastía de reducción con pedículo inferior / Pyoderma gangrenosum secondary to reduction mammoplasty

Background: Pyoderma gangrenosum (PG) is an uncommon inflammatory and ulcerative disorder of the skin. It may be associated with immune deficiencies. It is usually located in the extremities and its appearance after reduction mammoplasty is exceptional. We report three patients with the disease. A 54 years old female subjected to a bilateral reduction mammoplasty with an inferior pedicle. She developed a pyoderma gangrenosum and was treated with systemic steroids and local application of Dapsone with remission of lesions and healing after one month of evolution. A 23 years old women subjected to the same surgical procedure, which developed a wound dehiscence with ulcerative lesions, was treated with steroids and Azathioprine, reducing the local inflammation but leaving a severe esthetic sequel. A 21 years old woman subjected to the same surgical procedure, develop bilateral wound dehiscence and ulcerative lesions, she was treated with steroids and antimicrobials achieving a secondary healing...

Introducción: El Pioderma Gangrenoso es una enfermedad poco frecuente, de difícil diagnóstico y manejo. Puede estar asociado a otras enfermedades, en donde la alteración de la respuesta inmune es común. Su presentación por lo general corresponde a lesiones ulceradas de la piel ubicadas con frecuencia en las extremidades, siendo su presentación en las mamas excepcional; y mucho más rara su relación post mamoplastía de reducción. Métodos: Se realizó una revisión de pacientes sometidas a mamoplastía de reducción con pedículo inferior, asociadas al desarrollo de pioderma gangrenoso post-quirúrgico, durante un período comprendido entre los años 2000 y 2011. Resultados: Se recolectaron 3 pacientes, se describen sus casos clínicos con respecto a esta ubicación, analizando su presentación, evolución y manejo de la enfermedad. Discusión: La presentación del pioderma gangrenoso post-quirúrgico en relación a la mamoplastía de reducción comparte ciertas similitudes, las cuales deben orientar al diagnóstico precoz y de esta forma evitar manejos erróneos que pueden ser deletéreos, secuelantes e incluso potencialmente mortales...

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.

Biological therapy for pyoderma gangrenosum / Terapia biológica no tratamento do pioderma gangrenoso

INTRODUCTION: pyoderma gangrenosum (PG) is a rare and severe neutrophilic dermatosis associated with inflammatory bowel disease (IBD) and other systemic diseases such as rheumatoid arthritis and hematological malignancies. Diagnosis is based on clinical criteria and exclusion of other skin disorders. There is no gold standard for the treatment of PG; traditionally intravenous corticosteroids are used, but recently the use of drugs that inhibit tumor necrosis factor alpha (TNF-alpha) has changed the management of PG, showing great effectiveness. CASE REPORT: female patient, 23 years old, diagnosed with severe nonspecific ulcerative colitis (UC) three years ago, undergoing treatment with oral mesalamine and azathioprine. She developed PG fourteen days after hospital discharge; hospitalization was due to worsening of intestinal disease symptoms. She was successfully treated using biological therapy after unfavorable evolution with corticosteroid therapy. CONCLUSION: PG, a rare extraintestinal manifestation of IBD of difficult resolution that has significant impact on patient quality of life. The use of biological therapy for PG has higher efficacy in the treatment of patients decreasing wound healing time and return to daily activities. (AU)

INTRODUÇÃO: pioderma gangrenoso (PG) é uma rara e grave dermatose neutrofílica associada a doença inflamatória intestinal (DII) e a outras doenças sistêmicas como a artrite reumatoide e neoplasias hematológicas. O diagnóstico é baseado em critérios clínicos e exclusão de outras desordens da pele. Ainda não há padrão ouro para o tratamento do PG, tradicionalmente usa-se corticoides endovenosos, porém recentemente o uso de fármacos inibidores do fator de necrose tumoral alfa (TNF-alfa) tem mudado o manejo do PG mostrando grande efetividade. RELATO DO CASO: paciente feminina, 23 anos, com diagnóstico de retocolite ulcerativa inespecífica (RCUI) severa há três anos, em tratamento com mesalazina oral e azatioprina. Evoluiu apresentando PG quatorze dias após alta hospitalar, por internamento devido agudização dos sintomas intestinais da doença. Foi tratada com sucesso usando terapia biológica após evolução desfavorável com corticoide. CONCLUSÃO: PG, uma rara manifestação extraintestinal das DII, é de difícil resolução e com significante interferência na qualidade de vida dos pacientes. O uso de terapia biológica no PG vem mostrando melhor eficácia no tratamento desses doentes com diminuição do tempo de cicatrização das feridas e retorno às atividades diárias. (AU)

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.

Pioderma gangrenoso de la mama: reporte de un caso y revisión de la literatura / Pyoderma gangrenosum of the breast: a case report and literature review

El pioderma gangrenoso es una rara enfermedad inflamatoria que se caracteriza por una necrosis dolorosa de la piel que no cuenta con un tratamiento gold standard. Generalmente se asocia a enfermedades sistémicas, pero también se puede presentar después de procedimientos quirúrgicos. El diagnóstico es por exclusión, por esta razón es importante el estudio de enfermedades sistémicas e infecciones de la piel. El pioderma gangrenoso de la mama es un fenómeno poco frecuente, sólo se han reportado algunos casos. A continuación presentamos un caso de pioderma gangrenoso de la mama post reducción mamaria.

Pyoderma gangrenosum is a rare inflammatory disease characterized by a painful skin necrosis, and does not have a gold standard treatment. Usually associated with systemic diseases, may occur after surgical procedures. Diagnosis is made by exclusion, therefore it is important to rule out systemic diseases and infections of the skin. Pyoderma gangrenosum of the breast is a rare phenomenon, only few cases have been reported. We present a case of pyoderma gangrenosum of the breast post breast reduction.

Pioderma Gangrenoso: apresentação clínica de difícil diagnóstico / Pyoderma gangrenosum: a clinical manifestation of difficult diagnosis

Pioderma gangrenoso é uma dermatose cutânea ulcerativa incomum, associada a uma variedade de doenças sistêmicas, incluindo doença inflamatória intestinal, artrites, neoplasias hematológicas, hepatites e aids. A sua patogênese é desconhecida. O diagnóstico geralmente é baseado em evidências clínicas e confirmado com a exclusão das outras etiologias de lesões ulceradas cutâneas. Relatamos um caso de PG com ulcerações extensas com boa resposta ao tratamento.

Pyoderma gangrenosum is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases including inflammatory bowel disease, arthritis, hematological malignancies, hepatitis and acquired immunodeficiency syndrome (AIDS). The pathogenesis of pyoderma gangrenosum remains unknown. Its diagnosis is usually based on clinical evidence and confirmed through a process of elimination of the other possible causes of cutaneous ulcers. This report describes a case of pyoderma gangrenosum with extensive ulceration that responded well to treatment.

Pioderma gangrenoso en paciente con colitis ulcerosa tratada con infliximab / Pyoderma gangrenosum in a patient with ulcerative colitits treated with infliximab

El pioderma gangrenoso (PG) es una dermatitis neutrofílica, que en algunos casos puede ser severa y de difícil manejo. Presentamos el caso de una paciente con múltiples lesiones de PG asociadas a colitis ulcerosa, en la cual, por severidad del cuadro, se optó por adicionar infliximab al tratamiento habitual. La respuesta clínica fue excelente y rápida tras la primera dosis de infliximab, pese a que recibió solo dos de las tres dosis recomendadas habitualmente. La mejoría cutánea y digestiva se ha mantenido un año después de este tratamiento. Infliximab ha demostrado ser, en este y otros reportes, una herramienta muy útil, especialmente en casos de compromiso severo como en nuestra paciente. Se requiere mas evidencia aún para comprobar en cuáles pacientes podría ser beneficioso. Se presenta este caso por su severidad y la rápida y sostenida respuesta obtenida con infliximab.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis and in some cases can be severe and difficult to manage. We report the case of a patient with multiple lesions of PG associated with ulcerative colitis. Due to the severity of the clinical presentation treatment with infliximab was added to standard therapy. Clinical response was excellent and fast after the first dose of infliximab, although ha received only two of the three doses usually recomended. Skin and digestive improvement has been maintained 1 year after treatment. Infliximab has proven, in this and other reports, as a very useful tool in the treatment of PG, especially in cases of severe involvement as in our patient. More evidence is required to prove in which patients with PG infliximab could beneficial. We present this clinical case because of its severity and the rapid and susteined response obtained with infliximab.

Pioderma gangrenoso de rápida evolución / Pyoderma gangrenosum rapidly evolving

Se presenta caso de mujer adulta con múltiples lesiones ampollosas de 1 semana de evolución, acompañada de fiebre. Presentaba antecedentes de rectorragias ocasionales desde hace varios años. La colonoscopía detectó la presencia de Enfermedad inflamatoria intestinal, que se confirmó con la anatomía patológica. La biopsia de piel fue compatible con pioderma gangrenoso. La paciente mejoró con prednisona y aziatropina.

A case report of adult female with multiple bullous lesions of 1 week duration, accompanied by fever. She had a history of occasional rectal bleeding for several years. The colonoscopy detected the presence of inflammatory bowel disease, which was confirmed by pathology. Skin biopsy was consistent with pyoderma gangrenosum. The patient improved with prednisone and aziatropina.

Pyoderma Gangranosum of the Penis

We report a patient who developed pyoderma gangrenosum in the penis with invasion of the distal urethra. The patient was treated with prednisolone and thalidomide, followed by a reconstructive surgical repair using a scrotal island flap. We report this case with a brief review of the literature.