Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases

Abstract Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

Clinicopathological features and prognosis of pyoderma gangrenosum in Korea: A single centre, retrospective, observational study over 20 years

Background: Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum. Aims: We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum. Methods: In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999–2019. Results: Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0–9.0 months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence. Limitations: This study was a single-centre study with a retrospective design. Conclusion: Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum.

Prevalence of skin lesions in a sample of Brazilian patients with inflammatory bowel disease

SUMMARY OBJECTIVE: Inflammatory bowel diseases may have extra intestinal manifestations such as those affecting the skin. This study aimed to study skin manifestations in a cohort of Brazilian patients with inflammatory bowel diseases. METHODS: Epidemiological and clinical data were obtained through a cross-sectional study of 70 inflammatory bowel diseases patients and a control group comprising 50 healthy individuals. All patients were subjected to dermatological examination and photography of skin lesions. RESULTS: Out of the 70 inflammatory bowel diseases patients, 50 had ulcerative colitis and 20 had Crohn's disease. Skin lesions occurred in 95.7% of the inflammatory bowel diseases patients and in 88% of individuals in the control group (p=0.001). Alopecia (p<0.0001), xerosis (p=0.03), striae (p=0.02), and acne (p=0.04) were more common in inflammatory bowel diseases patients than in the control group. Alopecia was more frequent in females (p=0.01) than in males. Two male patients, one with ulcerative colitis and the other with Crohn's disease, had pyoderma gangrenosum. Erythema nodosum was not observed in both groups. CONCLUSION: There was a high prevalence of skin lesions in the Brazilian inflammatory bowel diseases patients. Additionally, alopecia, xerosis, striae, and acne were more common in patients with inflammatory bowel diseases than in those in the control group.

Analysis of clinical characteristics of 36 patients with pyoderma gangrenosum and comparison between the PARACELSUS score and Delphi criteria for their diagnosis / 中华皮肤科杂志

Objective:To analyze clinical characteristics, treatment, and outcomes of 36 patients with pyoderma gangrenosum, and to compare and evaluate the applicability and consistency between the PARACELSUS score and Delphi criteria.Methods:From January 2000 to January 2022, clinical data were collected from 36 patients who were diagnosed with pyoderma gangrenosum in the Hospital of Dermatology, Chinese Academy of Medical Sciences. The PARACELSUS score and Delphi criteria were applied to their diagnosis, and the kappa test was used to evaluate the consistency between the two diagnostic criteria.Results:Among the 36 patients, 6 (16.67%) had definite precipitating factors before the onset, and 31 (86.11%) exhibited lesions with different degrees of pain. Ulcerative lesions predominatd in 31 (86.11%) patients, which mostly involved the lower extremities, while 16 (44.44%) presented with multiple lesions all over the body. Four (11.11%) patients were complicated by inflammatory bowel disease, and 3 (8.33%) with inflammatory arthritis. Glucocorticoids, Tripterygium wilfordii, and cyclosporin were the main systemic treatment options, and tumor necrosis factor-α antagonist was used in 8 (22.22%) patients. Twenty-two (64.71%) and 17 (50.00%) patients were diagnosed with pyoderma gangrenosum using the PARACELSUS score and Delphi criteria, respectively. The kappa test showed moderate agreement between the two diagnostic criteria (kappa value = 0.47, 95% CI: 0.19 - 0.76, P = 0.004) . Conclusions:Classic ulcerative subtype was the major subtype in the patients with pyoderma gangrenosum, who were usually complicated by inflammatory bowel disease and inflammatory arthritis. Glucocorticoids and immunosuppressants were the main therapeutic drugs. The PARACELSUS score and Delphi criteria focus on different aspects of pyoderma gangrenosum, and the PARACELSUS score is recommended in the context of absence of typical histopathological manifestations.

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.

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.

Four Cases of the Rare Skin Manifestation: Monoclonal-Proteinemia / 罕见病研究

The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.

Skin manifestations associated with systemic diseases - Part I

Abstract The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

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.

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.

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 Arising De Novo Over an Unusual Site: A Case Report

Summary@#Pyoderma gangrenosum (PG) of the breast is a rare rapidly progressive neutrophilic dermatosis, which usually co-exists with severe underlying systemic conditions. A woman presented with a non-healing ulcer over her right breast with characteristic sparing of nipple-areola complex (Bork-Baykal phenomenon). It was diagnosed as pyoderma gangrenosum on the basis of clinico-pathological correlation and managed successfully with systemic corticosteroids and anti-inflammatory drugs along with wound care. The diagnosis and treatment of PG is challenging particularly at unusual sites given the paucity of robust clinical evidence and lack of consensus opinion regarding specific management guidelines. It is imperative that PG is considered as a clinical diagnosis in any patient with enlarging, sterile, necrotic lesions unresponsive to appropriate antibiotics. Early recognition of PG at rare locations can prevent devastating sequelae such as over-zealous surgical debridement and deep tissue infections associated with a chronic open wound leading to severe cosmetic morbidity.

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.

Proline-serine-threonine phosphatase interacting protein 1-associated autoinflammatory diseases / 中华实用儿科临床杂志

In addition to the pyogenic arthritis, pyoderma gangrenosum and acne(PAPA) syndrome, autoinflammatory diseases caused by mutations in the proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) gene also include a group of clinical syndromes such as PSTPIP1-associated myeloid-related proteinemia inflammatory(PAMI) and pyoderma gangrenosum, acne, and hidradenitis suppurativa(PASH) syndrome.In this paper, the expanded spectrum and clinical characteristics of PSTPIP1 related autoinflammatory diseases were reviewed, so as to deepen clinicians′ understanding of this disease, facilitate early diagnosis, and finally improve the prognosis of patients.

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.

Pyoderma gangrenosum in a patient with type 2 diabetes: a case report / Pioderma gangrenosa en un paciente diabético tipo 2: reporte de un caso

We are presenting a case of patient with type 2 diabetes mellitus that was diagnosed with a soft tissue infection in the lower extremity. This was initially treated as cellulitis and antibiotic treatment was initiated. Due to a poor clinical response, the diagnosis of pyoderma gangrenosum was proposed as part of the differential diagnosis. Skin biopsies and pathology confirmed the diagnosis of pyoderma gangrenosum that had a satisfactory response to steroid treatment

Se presenta el caso de una paciente diabética tipo 2 que inicialmente fue diagnosticada como una celulitis complicada del miembro inferior izquierdo, pero ante la pobre respuesta con el tratamiento antibiótico, se consideró el diagnóstico de pioderma gangrenosa, confirmado anatomopatológicamente, con respuesta satisfactoria al tratamiento esteroideo tópico

An unusual association of pyoderma gangrenosum with ulcerative colitis and thyrotoxicosis successfully treated with infliximab: a case report

Pyoderma Gangrenosum (PG) is a rare chronic immune-mediated inflammatory dermatosis manifested as painful skin ulceration, commonly affecting the lower limbs. The pathogenesis of the disease is complex. Abnormalities in neutrophil function, dysregulation of the innate immune system, and Tumor Necrosis Factor (TNF) were postulated. An underlying associated systemic disease was reported in more than 50% of PG patients, including inflammatory bowel disease, rheumatoid arthritis, and malignancies, with few cases reported an associated thyroid disease. Authors report a case of extensive PG associated with both ulcerative colitis and thyrotoxicosis co-morbidities not mentioned before in one patient. The patient was successfully treated with the anti-TNF alpha infliximab.

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

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