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)

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.

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.

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 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 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...

Perfuração de septo nasal em paciente com pioderma gangrenoso / Nasal septum perforation in patient with pyoderma gangrenosum

Introdução: A cocaína é extraída das folhas do arbusto da coca (Erythroxylon coca), podendo ser consumida de várias formas, mas o modo mais comum é pela aspiração da droga, sendo absorvida pela mucosa nasal, causando vasoconstricção, levando o seu uso crônico à perfuração de septo nasal. Pioderma gangrenoso é uma doença inflamatória rara, idiopática que se caracteriza pela presença de úlceras destrutivas principalmente em membros inferiores. Seu diagnóstico clínico é muitas vezes de exclusão. Objetivo: Descrever a raridade de associação entre Pioderma Gangrenoso e cocaína. Relato de Caso: E. A., 27 anos, com apresentação atípica de Pioderma Gangrenoso com perfurações de septo nasal e palato duro usuária de grande quantidade de cocaína, sendo necessário diferenciar qual patologia causou esse dano. Comentários Finais: Além da necessidade dessa diferenciação, apenas existem três casos relatados na literatura, envolvendo o Pioderma Gangrenoso complicado com perfuração de septo nasal em usuários de cocaína...

Introduction: The cocaine is obtained from the leaves of the coca (Erythroxylon coca). It can be used in many ways, but the most common is the drug inhalation. The Cocaine also causes vasoconstriction at nasal mucous membrane and its chronic use can cause necrosis and nasal septum perforation. Pyoderma gangrenosum is an uncommon idiopathic disease characterized by ulcerations, usually observed on the legs. Its diagnosis is most common an exclusion of others diseases. So far, there is no specific treatment based on evidence by randomized controlled trials. Objective: Describe the rare association between Pyoderma gangrenosum and cocaine. Case Report: E. A., 27-year-old woman with destruction of nasal septum and palate who has been using a big amount of cocaine, been necessary note the difference from which disease cause de damage. Final Comments: Also there are only three cases of Pyoderma gangrenosum complicated with nasal septum perforation in cocaine users...

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.

Pyoderma gangrenosum as a initial manifestation of ulcerative proctocolitis

Pyoderma gangrenosum is a rare inflammatory skin condition characterized by progressive and recurrent skin ulceration of destructive course. It is usually associated with rheumatoid arthritis, paraproteinemia, myeloproliferative diseases and inflammatory bowel diseases, especially non-specific ulcerative proctocolitis. In these situations, skin lesions are described as concurrent with the intestinal condition. However, reports on pyoderma gangrenosum preceding intestinal findings are less frequent. The authors describe a case of a woman with febrile condition associated with skin lesions diagnosed by biopsy as pyoderma gangrenosum. Two weeks later, she developed diarrhea, arthralgia and sepsis, being diagnosed as ulcerative proctocolitis. After the administration of the treatment for ulcerative proctocolitis, she showed improvements in sepsis care, remission of diarrhea and regression of skin lesions. This case highlights the importance of considering pyoderma gangrenosum as a manifestation associated with inflammatory bowel disease, regardless of its timing in relation to intestinal symptoms. (AU)

Pioderma gangrenoso é uma forma de inflamação cutânea, caracterizada por ulceração progressiva e recorrente da pele, com curso destrutivo. Geralmente é associada à artrite reumatoide, paraproteinemia, doenças mieloproliferativas e doença inflamatória intestinal, em especial retocolite ulcerativa inespecífica. Em tais casos, as lesões cutâneas são descritas concomitantes ao quadro intestinal, porém, relatos com descrição de pioderma gangrenoso precedendo achados intestinais são menos frequentes. Os autores relatam caso de mulher com quadro febril associado a lesões cutâneas diagnosticadas por biópsia como pioderma gangrenoso. Duas semanas depois, apresentou diarreia, artralgia e sepse sendo diagnosticada retocolite ulcerativa. Com o tratamento para retocolite ulcerativa apresentou melhora do quadro séptico, remissão da diarreia e regressão das lesões cutâneas. Este caso enfatiza a importância em considerar o pioderma gangrenoso como manifestação associada à doença inflamatória intestinal, independente de sua temporalidade em relação aos sintomas intestinais. (AU)

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.

Atypical pyoderma gangrenosum following total knee replacement surgery - First report in dermatologic literature / Pioderma gangrenoso atípico após artroplastia total do joelho: primeiro relato na literatura dermatológica

An atypical pyoderma gangrenosum mimicking a post surgical cutaneous and subcutaneous infection is being reported for the first time in international literature after knee replacement surgery with the operation site being the starting point of pyoderma gangrenosum. Orthopedic surgeons and general surgeons should be aware of the existence of this disease and its association with surgical trauma.

Um pioderma gangrenoso atípico, similar a uma infecção cutânea e subcutânea pós-operatório, está sendo relatado pela primeira vez na literatura mundial, depois de realização de artroplastia do joelho, tendo como foco inicial o local da cirurgia. Cirurgiões ortopédicos e gerais devem estar cientes da existência dessa doença e de sua associação ao trauma cirúrgico.

Pioderma granulomatoso superficial: relato de caso de variante rara do pioderma gangrenoso / Superficial granulomatous pyoderma: report of a case of an uncommon variant of pyoderma gangrenosum

O pioderma gangrenoso é doença cutânea inflamatória rara, idiopática. Afeta principalmente adultos; apenas cerca de 4 por cento dos casos são diagnosticados em crianças e adolescentes. Existem quatro formas clínicas de pioderma gangrenoso: ulcerativa, pustular, bolhosa e vegetante (pioderma granulomatoso superficial). O pioderma granulomatoso superficial é considerado a forma mais benigna e incomum da doença. Em pacientes submetidos a manipulação cirúrgica, uma eventual manifestação do pioderma gangrenoso ocorre nos locais de intervenção. Relata-se o caso de criança de cinco anos de idade, vítima de queimadura, que apresentou pioderma granulomatoso superficial sobre áreas doadoras de enxertos.

Pyoderma gangrenosum is a rare idiopathic skin disease. It affects mainly adults, and only 4 percent of the cases are diagnosed on children and adolescents. There are four clinical forms of pyoderma gangrenosum: ulcerative, pustular, bullous, and vegetative (superficial granulomatous pyoderma). Superficial granulomatous pyoderma is considered the most benign and uncommon form of the disease. Patients who have undergone surgical procedures may occasionally present pyoderma gangrenosum manifestations on the surgical site. A case of a five-year-old child, victim of burn, who presented superficial granulomatous pyoderma on the skin graft donor sites is reported.

Pyoderma gangrenosum: a clinical observation in an adult Nigerian

Case Definition: Pyoderma gangrenosum (PG) is a destructive; necrotizing non-infective ulceration of the skin of unknown aetiology and rarely reported amongst Nigerians. It is believed to be a reactive inflammatory dermatosis and partly a spectrum of neutrophilic dermatoses. PG usually starts as a painful nodule or pustule that gradually becomes turgid and breaks down to form a progressively enlarging ulcer; with raised tender; undermined violeacous borders. These ulcers may occur singly or in multiples; usually with a prediliction for the lower limbs but may occur elsewhere. It is associated with several medical disorders but can also occur spontaneously in 10-30of cases.1 PG is a diagnosis of exclusion2.Objective: The atypical presentation of Pyoderma gangrenosum in our patient from a typical hot humid tropical climate where other causes of cutaneous ulceration may simulate or mimic PG and must be excluded was the reason for revisiting this topic. A case of idiopathic pyoderma gangrenosum in a 29 years-old Nigerian male is herein discussed alongside important differentials of tropical cutaneous ulcerations that could mimic it

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

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

Pioderma periileostómico: caso clínico / Peri-ileostomic pyoderma gangrenosum: report of one case

We report a 39 years old woman with Ulcerative Colitis unresponsive to medical treatment. A total colectomy with Brooke ileostomy was performed, as the first operation. After six weeks she developed a peri-ileostomic Pyoderma Gangrenosum. She was treated by a multidisciplinary team, and after seven months with local treatment and systemic medication, the skin healed. Her bowel transit was reconstructed later with an ileoanal pouch and she was reinserted into her previous job (Rev Méd Chile 2004: 132: 747-9).

Pioderma gangrenoso postcesária / Post cesarean section pyoderma gangrenosum

Se describen dos casos de pioderma gangrenoso postquirúrgico. Se trata de dos mujeres jóvenes que se presentan con lesiones ulcerativas rápidamente progresivas, postcesárea y que responden rápidamente al tratamiento esteroidal. Se discuten los hallazgos clínicos, etiología, diagnósticos diferenciales con otras úlceras cutáneas y el tratamiento

A propósito de enfermedad de Buerger: un caso / A propos of Buerger's disease: presentation of a case

Paciente masculino de 56 años de edad que en diciembre de 1994 inicia cuadro clínico con disminución de la sensibilidad en segundo, tercero y cuarto dedos de mano izquierda, agregándose parestesias, mialgias y dolor ocasional a lo largo del miembro torácico izquierdo; tornándose progresivo con disminución del llenado capilar y fenómeno de Raynaud, con posterior formación de ampollas y salida de líquido purulento y fétido. Se realizó el diagnóstico de piodermia gangrenosa. Se le practica flebografía que muestra trombosis axilar y subclavia izquierda; las arteriografías de miembro torácico izquierdo evidenciaron isquemia importante en mano; el miembro torácico derecho estaba normal y el pélvico derecho con disminución de la circulación en pierna y pie. Se decide efectuar amputación de falanges distal y media del segundo al quinto dedo, debido a que, a pesar del tratamiento, la necrosis continuó avanzando. El diagnóstico establecido con base en el estudio de histopatología resultó compatible con enfermedad de Buerger, una enfermedad oclusiva inflamatoria poco común, que involucra arterias y venas de pequeño y mediano calibre de las extremidades, y que afecta a pacientes con antecedentes de tabaquismo crónico.

Pioderma gangrenoso: una enfermedad compleja, revision y actualización / Pyoderma gangrenosum

El propósito de este artículo es una puesta al día de los criterios diagnóstico y evaluación de los diferentes tratamientos de pioderma gangrenoso. El P.G. tiene cuatro formasa clínicas y variantes histológicas. Está asociado frecuentemente a enfermedades sistémicas. Hay diferentes clases de tratamiento para esta enfdermedad, ambos local y sistémico de acuerdo al tipo de P. G