Pioderma gangrenoso. Presentación de un caso

El pioderma gangrenoso es una enfermedad inflamatoria, poco común, de etilogía desconocida, caracterizada por la infiltración neutrófila estéril de la dermis, que puede o no estar asociada con enfermedades sistémicas. Su descripción clásica es la presencia de una o más lesiones ulceradas cutáneas, dolorosas, de aspecto infeccioso, bordes irregulares, socavados y con una desmesurada respuesta al trauma local denominado patergia. Se presenta el caso de una paciente de 58 años de edad, con lesiones en piel de dos meses de evolución y varios tratamientos previos sin mejorar. Al ser reevaluada, fue diagnosticado y tratado su caso como un pioderma gangrenoso con evolución satisfactoria.

Pyoderma gangrenosum is a rare inflammatory disease of unknown etiology characterized by sterile neutrophilic infiltration of the dermis, which may or may not be associated with systemic disease. Its classic description is the presence of one or more ulcerated skin lesions, painful, with an infectious appearance, irregular edges, undermined and with an excessive response to local trauma called pathergy. The case of a 58-years-old patient is presented, with skin lesions of two months' evolution and several previous treatments without improvement. Upon reassessment, her case was diagnosed and treated as pyoderma gangrenosum with satisfactory evolution.

Pioderma gangrenoso: atualização e orientação / Pyoderma gangrenosum: update and guidance

Introdução: O pioderma gangrenoso (PG) é uma doença neutrofílica, rara, porém de consequências danosas. O Capítulo de Feridas da Sociedade Brasileira de Cirurgia Plástica (SBCP) foi instado a compilar as melhores práticas, tanto diagnósticas como terapêuticas, junto às Sociedades Brasileiras de Dermatologia e Reumatologia para um melhor esclarecimento dos seus membros. Métodos: Ampla revisão de artigos publicados na literatura médica e compilação das novas diretrizes de diagnóstico e tratamento por dois membros indicados por cada uma das Sociedades Brasileiras de Cirurgia Plástica, Dermatologia e Reumatologia. Resultados: O PG deixou de ser uma doença de exclusão, tendo os critérios diagnósticos bem definidos e a orientação terapêutica delineada pelos autores, incluindo o uso de terapia biológica. Conclusão: O PG permanece desafiador, mas sistematizar a investigação e o uso dos novos medicamentos, bem como o manejo das feridas, abre novas perspectivas, interferindo na fisiopatologia de modo positivo, com maior precocidade e menos efeitos colaterais do que a terapia imunossupressora de forma isolada.

Introduction: The pyoderma gangrenosum (PG) is a neutrophilic disease, rare but with a poor outcome. The Capitulum of Wound treatment of the Brazilian Society of Plastic Surgery (SBCP) promoted a discussion with the Brazilian societies of Dermatology and Rheumatology to extract the best procedures in diagnostic and treatment. Methods: Broad review of published articles related to the subject and compilation of guidelines of diagnostic and treatment by two advisors of each involved society, plastic surgery, dermatology and rheumatology. Results: The PG is not an exclusion disease anymore, with well defined criteria for its diagnostic and literature based treatment, refined by the authors, including the use of biological therapies. Conclusion: The PG remains challenging, but systematizing the investigation and the use of new drugs has opened a new horizon of treatments, interfering in the pathophysiology in a positive manner with fewer side effects than immunosuppressive therapy alone.

Presentación cutánea atípica de histoplasmosis diseminada en paciente con VIH/sida / Atypical cutaneous presentation of disseminated

La histoplasmosis diseminada es una infección oportunista frecuente en los pacientes VIH/sida. Frecuentemente se encuentra compromiso cutáneo, siendo las formas clínicas más comunes el aspecto moluscoide, variceliforme y papulonecrótico.Se presenta el caso de una paciente con una forma cutánea atípica de histoplasmosis diseminada similar a pioderma gangrenoso cuyo diagnóstico fue realizado mediante escarificación.Es importante mantener la sospecha clínica en estas presentaciones atípicas y reforzar la utilidad de la escarificación de lesiones cutáneas como método rápido y efectivo para su diagnóstico.

Disseminated histoplasmosis is a frequent opportunistic infection in HIV/AIDS patients. Cutaneous manifestations are common, the most frequent are molluscum contagio-sum-like lesions, varicelliform eruption and papulo-necro-tic lesions.We present a case of an atypical disseminated cutaneous histoplasmosis with pyoderma gangrenosum-like ulcers, diagnosed by direct examination of tissue smear.It is important to have a high clinical suspicion of this atypical presentation and to emphasize the usefulness of skin smear as a simple and effective test to establish its diagnosis

Pioderma gangrenoso / Pyoderma gangrenosum

RESUMEN El pioderma gangrenoso (PG) es una enfermedad inflamatoria rara de causa desconocida, perteneciente al grupo de las dermatosis neutrofílicas, caracterizada por ulceraciones cutáneas dolorosas y recurrentes, asociada comúnmente con enfermedades autoinmunes o neoplasias, pero también aparece de forma independiente a estos trastornos. Existen varios subtipos: ulceroso o clásico, ampollar, pustuloso y vegetante. Se presenta una paciente femenina de 68 años de edad, con lesión ulcerosa cutánea, con cultivos bacteriológicos y micológicos negativos, biopsia cutánea que informó pioderma gangrenoso y exámenes complementarios para descartar enfermedades asociadas. No se evidenció asociación con enfermedades sistémicas.

ABSTRACT Pyoderma gangrenosum (PG) is a rare inflammatory disease of unknown cause, belonging to the group of neutrophilic dermatoses, characterized by painful and recurrent skin ulcerations and commonly associated with autoimmune diseases or neoplasms, although it can also occur independently of these disorders. Ulcerative or classic, bullous, pustular and vegetating forms are the different subtypes. We present a 68-year-old female patient with an ulcerative skin lesion, negative bacteriological and mycological cultures, a skin biopsy showing pyoderma gangrenosum and complementary examinations to rule out associated diseases. No association with systemic diseases was evidenced.

Osteomielitis aséptica craneal asociada a pioderma gangrenoso / Cranial aseptic osteomyelitis associated with pyoderma gangrenosum

Las dermatosis neutrofílicas (DN) constituyen un grupo heterogéneo de enfermedades. Excepcionalmente, las DN pueden acompañarse de acúmulos de neutrófilos estériles en otros tejidos distintos a la piel. Una paciente de 34 años consultó por una cefalea que no respondía al tratamiento analgésico. Una TAC demostró una lesión osteolítica a nivel parietal derecho, cuyo estudio histopatológico sugería una osteomielitis. Un año después del inicio de la cefalea desarrolló un pioderma gangrenoso en cara anterior de ambas piernas. Tras tratamiento con corticoterapia sistémica se resolvieron las lesiones cutáneas y la cefalea. La afectación ósea en las dermatosis neutrofílicas es excepcional. Habitualmente afecta a la población infantil en el contexto de una osteomielitis crónica recurrente multifocal (OCRM). Solo se han descrito dos casos en adultos, una paciente de 26 años, con una OCRM desde la infancia, y un varón de 67 años que desarrolló una osteomielitis aséptica en continuidad de un pioderma gangrenoso (AU)

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum (AU)

Cranial aseptic osteomyelitis associated with pyoderma gangrenosum.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

Tratamento exitoso de pioderma gangrenoso após mamoplastia redutora: relato de caso / Successful treatment of pyoderma gangrenosum after reducing mammoplasty: a case report

RESUMO Introdução: O pioderma gangrenoso é uma doença inflamatória rara caracterizada pela presença de lesões ulceradas. A etiologia é desconhecida, mas aparentemente relaciona-se com fatores imunológicos. Os autores relatam um caso de pioderma gangrenoso no pós-operatório de uma mamoplastia redutora, o tratamento e a importância do diagnóstico precoce para obtenção de um bom resultado estético. Métodos: Trata-se de uma paciente de 18 anos que desenvolveu pioderma gangrenoso após uma mamoplastia redutora. Os sintomas iniciais foram a formação de bolhas de coloração violácea e lesões ulceradas em ambas mamas. Suspeitou-se a doença e a paciente foi tratada prontamente com prednisolona. Foram realizadas culturas, biópsias e exames sanguíneos. Realizou-se terapia hiperbárica e suporte nutricional. Foram realizados curativos diários com fator de crescimento epidérmico. Após 60 dias de acompanhamento foi possível conseguir uma cicatrização adequada da feridas e bons resultados estéticos. Resultados: O diagnóstico precoce associado a uma terapia sistêmica imunossupressora e cuidados locais rigorosos foram fundamentais para a estabilização da doença. A associação com terapia hiperbárica e o suporte nutricional contribuíram para a epitelização das lesões e o controle do quadro clínico. A aproximação das bordas da ferida com sutura subdérmica e posterior sutura intradérmica foram possíveis devido à recuperação da derme e estabilização da doença. Conclusão: O diagnóstico precoce de pioderma gangrenoso é fundamental para conseguir a estabilização da doença. É possível atingir bons resultados estéticos baseados em 4 elementos: corticoide sistêmico precoce, curativos locais diários com fator de crescimento epidérmico, suporte nutricional e terapia hiperbárica.

Abstract Introduction: Pyoderma gangrenosum is a rare inflammatory disease characterized by the presence of ulcerated lesions. The etiology is unknown but apparently relates to immunological factors. The authors report a case of pyoderma gangrenosum in the postoperative period of a reduction mammoplasty, treatment, and the importance of early diagnosis to obtain a good aesthetic result. Methods: This is an 18-year-old patient who developed pyoderma gangrenosum after a reduction mammoplasty. The initial symptoms were the formation of violet blisters and ulcerated lesions in both breasts. The disease was suspected, and the patient was promptly treated with prednisolone. Cultures, biopsies and blood tests were performed. Hyperbaric therapy and nutritional support were performed. Daily dressings with epidermal growth factors were performed. After 60 days of follow-up, it was possible to achieve adequate wound healing and good aesthetic results. Results: Early diagnosis associated with systemic immunosuppressive therapy and rigorous local care were fundamental for disease stabilization. The association with hyperbaric therapy and nutritional support contributed to the lesion's epithelialization and the clinical picture control. The approximation of the wound edges with subdermal suture and the subsequent intradermal suture was possible due to the recovery of the dermis and stabilization of the disease. Conclusion: Early diagnosis of pyoderma gangrenosum is essential to achieve stabilization of the disease. It is possible to achieve good aesthetic results based on four elements: early systemic corticosteroids, daily local dressings with epidermal growth factor, nutritional support, and hyperbaric therapy.

O pioderma gangrenoso como diagnóstico diferencial de complicações isquêmicas e infecciosas após abdominoplastia: um relato de caso / Pyoderma gangrenosum as a differential diagnosis of ischemic and infectious complications after abdominoplasty: a case report

Introdução: A abdominoplastia é um procedimento com índice considerável de complicações, ainda que, em sua maioria, de bom prognóstico. Algumas complicações, entretanto, podem ser catastróficas, como a necrose extensa de pele e as complicações infecciosas graves. Dentre as causas incomuns de perda extensa de pele no pós-operatório, podemos citar o pioderma gangrenoso (PG), doença de curso crônico, recidivante, com comportamento imprevisível e de etiologia ainda desconhecida. No âmbito da cirurgia plástica, essa doença pode mimetizar clinicamente complicações pós-operatórias isquêmicas ou infecciosas, cujos tratamentos diferem por completo do tratamento do PG. Relato de Caso: Paciente feminina, 41 anos, previamente hígida, foi submetida à abdominoplastia associada à lipoaspiração e mamoplastia de aumento com colocação de próteses mamárias. Evoluiu com edema, calor hiperemia e dor em incisão de abdominoplastia, além de comprometimento clínico sistêmico. Submetida a desbridamentos cirúrgicos e tratamento sistêmico, com piora progressiva das lesões. Diante do insucesso dos tratamentos propostos, aventada a hipótese diagnóstica de pioderma gangrenoso. Conclusão: O PG, apesar de raro, deve ser aventado como diagnóstico diferencial em casos de complicações pós-operatórias com perda e necrose de pele que não respondem às medidas iniciais de tratamento, além de quadros aparentemente infecciosos que não respondem às terapias antibióticas adotadas.

Introduction: Abdominoplasty is a procedure with a considerable rate of complications, even though, for the most part, it has a good prognosis. Some complications, however, can be catastrophic, such as extensive skin necrosis and serious infectious complications. Among the unusual causes of extensive skin loss in the postoperative period, we can mention gangrenous pyoderma (PG), a chronic, recurrent disease with unpredictable behavior and an unknown etiology. In the field of plastic surgery, this disease can clinically mimic ischemic or infectious postoperative complications, whose treatments differ completely from the treatment of PG. Case Report: A 41-year-old female patient, previously healthy, underwent abdominoplasty associated with liposuction and breast augmentation with the placement of breast implants. The patient evolved with edema, hyperemia and pain in an abdominoplasty incision, in addition to systemic clinical involvement. She was submitted to surgical debridement and systemic treatment, with progressive worsening of the lesions. In view of the failure of the proposed treatments, the diagnostic hypothesis of gangrenous pyoderma was raised. Conclusion: PG, although rare, should be considered as a differential diagnosis in cases of postoperative complications with skin loss and necrosis that do not respond to initial treatment measures, in addition to apparently infectious conditions that do not respond to adopted antibiotic therapies.

Síndrome malformativo de manos y pies hendidos / Malformation of cleft hand and foot syndrome

RESUMEN Introducción: el síndrome malformativo de manos y pies hendidos, es un defecto congénito poco frecuente con patrón de herencia autosómico dominante, de forma simétrica. Presentación de caso: se presenta una lactante de tres meses que nace a las 36 semanas, con peso de 2 450 gramos, talla 50 cm, Apgar 8-9. Se detecta al nacimiento, hendidura mediana en ambas manos y pies con ectrodactília de 2do y 3er dedo de todos sus miembros, no otras malformaciones. El servicio de neonatología del hospital pediátrico provincial solicita valoración por genética clínica que después de un examen físico exhaustivo, llega al diagnóstico sindrómico. Es el primer caso de la provincia. Conclusiones: el método clínico es primordial para realizar el diagnóstico con la ayuda de los estudios radiológicos y clasificar el tipo de ectrodactília. Se mantiene con seguimiento multidisciplinario por ortopédicos, psicólogos, fisiatras y genetista clínico, quienes brindan un adecuado asesoramiento genético, así como la información de futuras operaciones que mejorarán la funcionalidad de sus miembros y elevarán su calidad de vida.

ABSTRACT Introduction: malformation of cleft hand and foot syndrome is a rare congenital defect with autosomal dominant inheritance pattern, symmetrical in shape. Case presentation: a three-month-old female infant born at 36 weeks, weighing 2450 grams, height 50 cm, Apgar 8-9, detected at birth median cleft in both hands and feet with ectrodactyly of the 2nd and 3rd fingers of all limbs, no other malformations. The neonatology service of the provincial pediatric hospital requests assessment by clinical genetics that after an exhaustive physical examination leads to the syndromic diagnosis, resulting in the first case in the province. Conclusions: considering of great value the clinical method to conduct the diagnosis with the help of radiological studies to be able to classify the type of ectrodactyly. The patient is kept with multidisciplinary follow-up by orthopedics, psychologists, physiatrists and clinical geneticist, providing adequate genetic counseling, as well as information on future operations that will improve the functions of her limbs and improve her quality of life.

Cuidados y tratamiento del pioderma gangrenoso periestomal. A propósito de tres casos / Care and treatment of peristomal gangrenous pyoderma. A study of three cases

El pioderma gangrenoso periestomal (PGP) es una enfermedad inflamatoria de la piel con evolución a úlcera dolorosa, muy poco frecuente, que rara vez se asocia con el carcinoma colorrectal.Su diagnóstico es diferencial puesto que puede confundirse con una infección de la piel, absceso, dermatitis de contacto, irritación periestomal o la extensión cutánea periestomal de una enfermedad inflamatoria intestinal (EII).Se presentan tres casos de pacientes intervenidos por carcinoma colorrectal con estoma intestinal, que desarrollaron PGP.Se elaboró un plan de cuidados y curas locales, empleando las taxonomías de la North American Nursing Diagnosis Association (NANDA), la clasificación de resultados de enfermería (NOC) y la clasificación de las intervenciones enfermeras (NIC).Los cuidados del estoma y la adecuación de dispositivos colectores se realizaron con suero fisiológico, pasta, polvos de ostomía y bolsa de dos piezas.Para el tratamiento local básico se utilizó suero fisiológico o la solución de lavado para la limpieza de herida, eosina acuosa (2%), alginato en fase exudativa y pomada con colagenasa en presencia de necrosis esfacelos.El tratamiento local específico (clobetasol propionato, tacrólimus o infiltración de triamcinolona acetónido) y sistémico (corticoterapia) se efectuó de forma secuencial tras el diagnóstico de PGP en función de la respuesta clínica a cada terapia.El primer caso se resolvió a los seis meses con buena respuesta a la infiltración local de triamcinolona. El segundo se recuperó a los 10 meses tras infiltración local con triamcinolona y prednisolona oral. El tercero no tuvo respuesta a los tratamientos locales ni a la corticoterapia sistémica, curándose después de la exéresis tumoral y metastásica con reubicación del estoma a los nueve meses.(AU)

Peristomal gangrenous pyoderma is an inflammatory skin disease with progression to painful ulcer, rare, and rarely associated with colorectal carcinoma.Its diagnosis is differential since it can be confused with skin infection, abscess, contact dermatitis, peristomal irritation or peristomal skin extension of an inflammatory bowel disease.We present three cases of patients operated for colorectal carcinoma with an intestinal stoma, who developed peristomal gangrenous pyoderma.A plan of local care and dressings was developed using the NANDA, NOC and NIC taxonomies.Stoma care and fitting of collecting devices were performed with saline solution, paste, ostomy powders and a two-piece bag.For the basic local treatment, physiological serum or washing solution was used for wound cleaning, aqueous eosin (2%), alginate in the exudative phase, and collagenase ointment in the presence of slough/necrosis.Specific local treatment (clobetasol propionate, tacrolimus, or triamcinolone acetonide infiltration) and systemic treatment (corticosteroid therapy) was given sequentially after the diagnosis of peristomal gangrenous pyoderma depending on the clinical response to each treatment.Case 1 resolved at six months with a good response to local triamcinolone infiltration. Case 2 resolved at 10 months after local infiltration with triamcinolone and oral prednisolone. Case 3 had no response to local treatments or systemic corticosteroid therapy, healing after tumour and metastatic excision with relocation of the stoma at nine months.(AU)

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)

Cranial aseptic osteomyelitis associated with pyoderma gangrenosum. / Osteomielitis aséptica craneal asociada a pioderma gangrenoso.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

Care and treatment of peristomal gangrenous pyoderma. A study of three cases. / Cuidados y tratamiento del pioderma gangrenoso periestomal. A propósito de tres casos.

Peristomal gangrenous pyoderma is an inflammatory skin disease with progression to painful ulcer, rare, and rarely associated with colorectal carcinoma. Its diagnosis is differential since it can be confused with skin infection, abscess, contact dermatitis, peristomal irritation or peristomal skin extension of an inflammatory bowel disease. We present three cases of patients operated for colorectal carcinoma with an intestinal stoma, who developed peristomal gangrenous pyoderma. A plan of local care and dressings was developed using the NANDA, NOC and NIC taxonomies. Stoma care and fitting of collecting devices were performed with saline solution, paste, ostomy powders and a two-piece bag. For the basic local treatment, physiological serum or washing solution was used for wound cleaning, aqueous eosin (2%), alginate in the exudative phase, and collagenase ointment in the presence of slough/necrosis. Specific local treatment (clobetasol propionate, tacrolimus, or triamcinolone acetonide infiltration) and systemic treatment (corticosteroid therapy) was given sequentially after the diagnosis of peristomal gangrenous pyoderma depending on the clinical response to each treatment. Case 1 resolved at six months with a good response to local triamcinolone infiltration. Case 2 resolved at 10 months after local infiltration with triamcinolone and oral prednisolone. Case 3 had no response to local treatments or systemic corticosteroid therapy, healing after tumour and metastatic excision with relocation of the stoma at nine months.

Miocardiopatia Dilatada após uso de Adalimumabe em paciente com Pioderma Gangrenoso / Dilated myocardiopathy after use of Adalimumab in a patient with pyoderma gangrenosum

Introdução: O pioderma gangrenoso é uma dermatose incomum, crônica e de apresentação clínica variável. Sua etiologia é indefinida, e seu tratamento não é bem estabelecido. O Adalimumabe é um anticorpo monoclonal humano contra o fator de necrose tumoral alfa (anti- TNF) que é raramente associado à disfunção cardíaca. Relato de Caso: Relatamos um caso de uma paciente com pioderma gangrenoso idiopático que, após duas doses de Adalimumabe, desenvolveu insuficiência cardíaca grave com miocardiopatia dilatada. Ela foi tratada com diurético de alça, betabloqueador e antagonista da enzima conversora da angiostensina. Após dois meses, encontrava-se sem queixas cardiorrespiratórias e com melhora da fração de ejeção. Conclusão: A miocardiopatia dilatada, apesar de ser manifestação rara do uso de fármacos anti -TNF deve ser considerada no diagnóstico diferencial de insuficiência respiratória após seu uso.

Introduction: Pyoderma gangrenosum is an unusual chronic dermatosis of mixed clinical presentation. Its etiology is unclear, and its treatment is not well established. Adalimumab is a human monoclonal antibody against the tumor necrosis factor alpha (anti TNF) which is rarely associated with cardiac dysfunction. Case Report: We report a case of a patient with idiopathic Pyoderma gangrenosum who developed severe heart failure with dilated myocardiopathy after two doses of Adalimumab. She was treated with loop diuretic, beta-blocker and angiostensin-converting enzyme antagonist. After two months, she was without cardiorespiratory complaints and with improvement in the ejection fraction. Conclusion: Dilated myocardiopathy, despite being a rare manifestation of the use of anti -TNF drugs should be considered in the differential diagnosis of respiratory failure after its use.

Pioderma Gangrenoso asociado a colitis ulcerosa en gestante / Pyoderma gangrenosum associated with ulcerative colitis in a pregnant woman

RESUMEN Introducción: El 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. Presentación de caso: Paciente femenina de la raza blanca de 26 años de edad con antecedentes de colitis ulcerosa y ahora con ocho semanas de gestación, que se presenta a consulta de gastroenterología del Hospital General Docente "Abel Santamaría Cuadrado", remitido de su área de salud, por presentar diarreas con flemas y sangre, además de lesiones nodulares, pápulas y vesículas múltiples con bordes irregulares, en miembros inferiores, superiores y región posterior del tronco, muy dolorosas. Conclusiones: La evolución de la colitis ulcerosa y la gestación se correlacionan con la actividad de la enfermedad en el momento de la concepción, de forma que la existencia de brote de actividad en este momento se ha asociado a un mayor riesgo de aborto y a una peor respuesta al tratamiento médico.

ABSTRACT Introduction: pyoderma gangrenosum is a rare chronic inflammatory dermatosis, which etiology and pathogenesis is still uncertain, although immunological factors and neutrophilic dysfunction seem to play an important role. In most cases, it occurs associated with non-infectious systemic disease, such as rheumatoid arthritis, hematological malignancy and inflammatory bowel disease. Case report: a 26 year-old white female patient with a history of ulcerative colitis and eight weeks pregnant, who presented herself to the gastroenterology office at Abel Santamaria Cuadrado General Teaching Hospital, she was referred from her health area, presenting diarrhea with phlegm and blood, in addition to nodular lesions, papules and multiple vesicles with irregular edges in lower limbs, upper limbs and posterior region of the trunk, very painful. Conclusions: the evolution of ulcerative colitis and pregnancy are correlated with the activity of the disease at the time of conception, so that the existence of an outbreak at this time has been associated with a higher risk of abortion and a worse response to medical treatment.

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.

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.

Pioderma gangrenoso paraneoplásico en una paciente con recaída de leucemia mieloide aguda / Paraneoplastic bullous pyoderma gangrenosum occurring in a patient with a relapse of an acute myeloid leukemia

Resumen El pioderma gangrenoso ampolloso fue descrito por primera vez en 1972. Se presenta el caso de una paciente con pioderma gangrenoso asociado a una recaída de leucemia mieloide aguda y se hace una revisión de la literatura sobre el tema.

Abstract Bullous pyoderma gangrenosum was first described by Perry in 1972. We present a case of a patient with paraneoplastic pyoderma gangrenosum associated to relapse of an acute myelogenous leukemia and we review the literature on pyoderma gangrenosum.