Pénfigo foliáceo de presentación inusual / Penphigus foliaceus of unusual presentation

Si bien las enfermedades ampollares autoinmunes son infrecuentes en la infancia y la juventud, es importante tenerlas en cuenta dentro del diagnóstico diferencial, junto con otras enfermedades inflamatorias o infecciosas de mayor prevalencia, para lograr un diagnóstico temprano y así indicar el tratamiento preciso y oportuno. El pénfigo foliáceo es una enfermedad ampollar poco común, salvo en la zona endémica de Brasil. Se presenta un caso de pénfigo foliáceo con eritrodermia como cuadro clínico con mala evolución (AU)

Although autoimmune bullous diseases are infrequent in childhood and youth, it is important to take them into account within the differential diagnosis, along with other more prevalent inflammatory or infectious diseases, to achieve an early diagnosis and thus indicate precise and timely treatment. Pemphigus foliaceus is a rare autoimmune bullous diseases, except in the endemic area of Brazil. This case report presents a patient with the unusual erythrodermic presentation of pemphigus with poor evolution (AU)

Câncer de Mama e Eritrodermia: Relato de Caso / Breast Cancer and Erythroderma: Case Report / Cáncer de Mama y Eritrodermia: Relato de Caso

Introdução: A eritrodermia esfoliativa é um evento raro que está associado a diversas doenças como psoríase, eczemas, neoplasias malignas, uso de medicamentos, entre outras. Relato do caso: Paciente de 63 anos apresentou quadro de exantema difuso iniciado em janeiro de 2018, evoluindo com descamação generalizada da pele e formação de úlceras, sentindo calafrios. Não referiu histórico de câncer na família. Na anamnese, não houve relato de nenhum tipo de eczema, doença de pele preexistente ou uso de plantas medicinais. Foi identificada neoplasia maligna de mama do subtipo molecular luminal A, posteriormente. Após mastectomia com linfadenectomia, houve apenas melhora parcial do quadro da eritrodermia. Atualmente, em uso de doxorrubicina. Conclusão: A paciente apresentou quadro clínico inicial condizente com o que se espera de eritrodermia, que pode estar associada ao surgimento de neoplasia maligna de mama. O presente relato é importante, pois pode auxiliar em diagnósticos diferenciais para a eritrodermia, mesmo na vigência de um quadro clínico atípico.

Introduction: Exfoliative erythroderma is a rare event associated with several diseases such as psoriasis, eczema, malignant neoplasms, medication use, among others. Case report: A 63-year-old patient presented diffuse rash that started in January 2018, evolving with generalized skin desquamation and ulcer formation, with chills. Did not report family history of cancer. In the anamnesis, there was no report of any type of eczema, preexisting skin disease or use of medicinal plants. Malignant breast cancer of luminal A molecular subtype was identified later. After mastectomy with lymphadenectomy, there was only partial improvement in erythroderma. Currently using doxorubicin. Conclusion: The patient presented an initial clinical condition consistent with what is expected from erythroderma, which may be associated with the emergence of malignant breast neoplasm. The present report is important because it can help in differential diagnoses for erythroderma, even in the presence of an atypical clinical case.

Introducción: La eritrodermia exfoliativa es un evento raro asociado con varias enfermedades como psoriasis, eccema, neoplasmas malignos, uso de medicamentos, entre otros. Relato del caso: Paciente de 63 años presentó una erupción cutánea difusa que comenzó en enero de 2018, que evolucionó con descamación generalizada de la piel y formación de úlceras, con escalofríos. No informó antecedentes familiares de cáncer. En la anamnesis, no hubo informes de ningún tipos de eccema, enfermedad cutánea preexistente o uso de plantas medicinales. La neoplasia de mama maligna del subtipo molecular luminal A se identificó más tarde. Después de la mastectomía con linfadenectomía, solo hubo una mejoría parcial en la eritrodermia. Actualmente usa doxorrubicina. Conclusión: La paciente presentó un cuadro clínico inicial consistente con lo que se espera de la eritrodermia, que puede estar asociada con la aparición de neoplasma maligno de mama. El presente informe es importante porque puede ayudar en los diagnósticos diferenciales de eritrodermia, incluso en presencia de un cuadro clínico atípico.

Síndrome de DRESS asociado al uso de anticonvulsivantes / DRESS syndrome associated to the use of anti-convulsiveness

Introducción: El síndrome Drug Reaction with Eosinophilia and Systemic Symptoms - DRESS, constituye una grave reacción adversa a medicamentos, principalmente a fármacos anticonvulsivantes. Objetivo: Describir la evolución clínica de un síndrome de DRESS en una paciente atendida en el Hospital Militar Central "Dr. Luis Díaz Soto". Caso Clínico: Paciente femenina de 27 años de edad con antecedentes patológicos personales de epilepsia. Tres meses luego de iniciada terapia con difenilhidantoína aparece fiebre, exantema maculopapular que progresa a eritrodermia exfoliativa, signos de daño hepático, adenopatías cervicales y eosinofilia. Se diagnosticó síndrome de DRESS secundaria al uso de anticonvulsivantes. Conclusiones: La evolución clínica resultó favorable, luego de la retirada del fármaco y la aplicación de esteroides por vía oral(AU)

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms - DRESS, syndrome constitutes a serious adverse reaction to medications, mainly anticonvulsant drugs. Objective: To describe the clinical evolution of DRESS syndrome in a patient treated at the Hospital Militar Central "Dr. Luis Díaz Soto". Case Report: 27-year-old female patient with a personal pathological history of epilepsy. Three months after initiation of diphenylhydantoin therapy, fever appeared maculopapular rash that progressed to exfoliative erythroderma, signs of liver damage, cervical adenopathies and eosinophilia. DRESS syndrome was diagnosed secondary to the use of anticonvulsants. Conclusions: The clinical evolution was favorable, after the withdrawal of the drug and the application of steroids orally(AU)

Erythroderma as first manifestation of squamous cell lung cancer: rare case report

Abstract Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Epidemiology of exfoliative dermatitis at the University of Santo Tomas Hospital (Department of Dermatology) from 2008-2012: A five-year review

@#INTRODUCTION: Exfoliative dermatitis is a potentially life- threatening inflammatory reaction that poses a significant risk for morbidity and mortality. Several underlying etiologies of this dermatologic condition include pre-existing dermatoses, drugs and malignancy. Although it is a common disease entity, local studies on exfoliative dermatitis published in literature are very limited. OBJECTIVE: The primary objective of this study is to determine the epidemiological profile of patients with exfoliative dermatitis diagnosed at University of Santo Tomas Hospital Dermatology department from January 2008 to December 2012. METHODS: Inpatient and outpatient clinical records of patients diagnosed and treated as exfoliative dermatitis were retrieved. The prevalence, clinical presentation, history of previous dermatoses or use of any drugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. RESULTS: A total of 67 patients were included in this retrospective study. The prevalence among patients with exfoliative dermatitis in this study was computed at 1 per 1000 dermatologic patients. The highest number of cases belonged to the group aged seventy-one to seventy-nine (25.4%) with a mean age of 56.62 years. There was a male predilection (65.7%). Clinical presentation of patients included pruritus, generalized scaling and erythema, accompanied by bipedal edema (41.8%), chills (22.4%), fever (T ≥ 38 °C), lymphadenopathies (6%) and joint pains (4.5%). Several etiologic factors of exfoliative dermatitis recorded were: pre-existing dermatosis (67.2%), idiopathic or undetermined causes (19.4%), drug-induced (10.4%) and malignancy (3%). CONCLUSION: Exfoliative dermatitis is a condition more commonly found in the older age group. Pre-existing dermatoses, drugs and malignancy are etiologic factors. The most common pre-existing dermatosis causing exfoliative dermatitis in this study is psoriasis while the most implicated drug is allopurinol.

Erythroderma as a manifestation of leprosy

Abstract: Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.

A Case of Exfoliative Dermatitis Induced by Phototherapy Secondary to Pustular Psoriasis / 대한피부과학회지

No abstract available.

Estudio clínico-histopatológico de las eritrodermias en un hospital universitario: 14 años de experiencia / Clinical-histopathological study of erythroderma in a university hospital: 14 years of experience

INTRODUCCIÓN: La eritrodermia es un síndrome inflamatorio cutáneo infrecuente caracterizado por compromiso eritematoso generalizado y descamación, de más del 90% de superficie cutánea total. OBJETIVO: Caracterizar clínica e histopatológicamente a los pacientes con eritrodermia en un hospital universitario chileno. METODOLOGÍA: Estudio retrospectivo, realizado en el Hospital Clínico Universidad de Chile, basado en revisión de fichas clínicas e informes histopatológicos de pacientes con eritrodermia, entre 2005 y 2018. Se evaluó edad, sexo y variables clínicas (co-morbilidades, síntomas, días de evolución, ingreso hospitalario, informe histopatológico, diagnóstico y evolución). RESULTADOS: Total de 28 pacientes, 18 hombres (64%), edad promedio 59 años. Causa más frecuente de eritrodermia fue dermatosis pre-exis-tentes, con 15 casos (54%), que incluyen: psoriasis 9 (32%), dermatitis de contacto 3 (11%), PRP 2 (7%), dermatitis atópica 1 (4%). A estas le siguen: reacción adversa medicamentosa 6 (21%), idiopática 6 (21%) y Síndrome de Sezary 1 (4%). CONCLUSIÓN: El presente estudio corresponde a la primera serie de eritrodermias realizada en Chile. Destacan las dermatosis preexistentes como la principal causa, lo que se correlaciona con la literatura.

INTRODUCTION: Erythroderma is an infrequent cutaneous inflammatory disorder characterized by generalized erythematous compromise and desquamation, of more than 90% of total cutaneous surface. OBJECTIVE: Clinical and histopathological cha-racterization of patients with erythroderma in a Chilean university hospital. METHODOLOGY: Retrospective study, performed at the University of Chile Clinical Hospital, based on review of clinical records and histopatho-logical reports of patients with erythroderma, between 2005 and 2018. Age, sex and clinical variables were evaluated (co-morbidities, symp-toms, days of evolution, hospital admission, histopathological report, diagnosis and evolu-tion). RESULTS: A total of 28 patients, 18 were men (64%), average age 59 years. Most frequent cause of erythroderma was pre-existing dermatosis, with 13 cases (52%), which included: psoriasis 9 (32%), contact dermatitis 3 (11%), PRP 2 (7%), atopic dermatitis 1 (4%). These are followed by adverse drug eruption 6 (21%), idiopathic 6 (21%) and Sezary syndrome 1 (4%). CONCLUSION: The present study corresponds to the first series of erythrodermas performed in Chile. The pre-existing dermatoses were the main cause of erythroderma, which coincides with other reports.

Wet wrap dressings as a rescue therapy option for erythrodermic psoriasis

Abstract: Wet wrap dressings provide an ancillary treatment option for erythrodermic patients. Wet wrap therapy consists of the application of topical corticosteroids on all affected surfaces, followed by the application of a warm moist cotton cloth with a dry cotton cloth on top. The advantages of this procedure include its safety, as well as the prompt and marked improvement of the erythroderma. For erythrodermic psoriasis, wet wrap dressings can serve as an important rescue therapy option. The purpose of this study is to report a case series of seven patients with erythrodermic psoriasis that responded promptly to wet wrap therapy prior to the initiation of systemic treatment.

Erythrodermic psoriasis and human immunodeficiency virus: association and therapeutic challenges

Abstract: Erythrodermic psoriasis is a rare but severe type of psoriasis that may be triggered by human immunodeficiency virus infection. We describe the case of a 65-year-old male patient with chronic psoriasis who presents an exacerbation of his condition over a period of two weeks. Because of the severity of his case and subsequent need for systemic therapy, human immunodeficiency virus enzyme immunoassay was performed and tested positive. He thus began antiretroviral therapy combined with acitretin, showing good clinical response after 8 weeks of treatment. There is little evidence regarding the management of erythrodermic psoriasis associated with HIV infection, so antiretroviral therapy and systemic retinoid remain as the first-line treatment.

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Abstract: Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Caracterización clínica y sociodemográfica de pacientes con psoriasis en un hospital universitario en Colombia / Clinical characterization and socio-demographic of patients with psoriasis at a university hospital in Colombia

Introducción y objetivo: La psoriasis es una enfermedad inflamatoria sistémica que afecta la piel, tiene predisposición genética y está mediada por mecanismos inmunológicos. Se presenta en 2% de la población occidental. En Colombia no hay datos epidemiológicos que permitan hacer una caracterización clínica. El objetivo de este estudio es conocer e identificar las prin-cipales variables clínicas y sociodemográficas de pacientes con diagnóstico de psoriasis en un Hospital Universitario de Colombia. Métodos: Es un estudio observacional, descriptivo, de corte transversal retrospectivo. La población estudio fueron pacientes de 13 a 90 años que ingresaron al Hospital Universitario de Santander entre 2012-2016.

Eritrodermia psoriática en un hombre joven: sospechar infección por VIH / Erythrodermic psoriasis in young man: suspect HIV infection

Resumen La infección por VIH puede presentarse con distintas manifestaciones cutáneas, que en algunas ocasiones son consideradas marcadores de infección. Una posible manifestación es la eritrodermia psoriática, que corresponde a una forma generalizada de psoriasis. Presentamos un caso clínico de un hombre joven en que se sospechó una infección por VIH por un cuadro de eritrodermia psoriática confirmada por biopsia, asociado a un sarcoma de Kaposi. Posteriormente, la infección por VIH fue confirmada por serología. Se manejó con terapia antirretroviral, con buena respuesta al mes de tratamiento. La eritrodermia psoriática se puede considerar un marcador cutáneo de infección por VIH cuando ocurre en pacientes previamente sanos o con psoriasis recalcitrante.

HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis.

Psoriasis: presentación de un caso y revisión bibliográfica / Psoriasis case presentation and review

Antecedentes: La psoriasis es una enfermedad inmune crónica con manifestaciones dermatológicas y articulares. Puede presentarse a cualquier edad pero ocurre con mayor frecuencia en dos picos: 20-30 años y 50-60 años. Tiene un componente genético fuerte porque aproximadamente el 30% de los pacientes tienen un familiar de primer grado con la enfermedad, y sus hijos pueden de-sarrollar artritis psoriásica sin alteraciones cutáneas. El paciente experimenta pocos periodos de remisión espontanea.Caso clínico: Mujer de 39 años, ama de casa del casco rural de Tela, diagnosticada con psoriasis hace 7 años. Acude a consulta en buen estado general sin secuelas psicológicas, con lesión en placa localizada en codo derecho con área afectada del 4% y eritrodermia psoriásica que cubre la totalidad del abdomen en un 9% de la supericie corporal. Reiere mejoría con los productos naturales. Actualmente con sobrepeso (27 IMC) y con hipertensión sistólica aislada (140/82 mmHg) sugerente de ateroesclerosis. Discusión: Se deben tomar en cuenta los aspectos psico-sociales y metabólicos que afectan la calidad de vida de estos pacientes, para tratarlos de forma inte-gral con un grupo multidisciplinario. El médico general puede manejar la psoriasis en casos concretos como las placas localizadas ≤ 5% del BSA, en los cuales el tratamiento indicado es tópico con corticoesteroides de alta potencia como primera línea. Para todo lo demás, el paciente con psoriasis debe ser evaluado por un dermatólogo.

Síndrome da pele escaldada estafilocócica no adulto. Relato de caso / Staphylococcal Scalded Skin Syndrome in adults: case report

A síndrome da pele escaldada estafilocócica é uma dermatose esfoliativa rara no adulto, que acomete cerca de 0,09 a 0,56 por milhão de habitantes. É causada por exotoxinas produzidas por Staphylococcus aureus e manifesta-se com a formação de lesões bolhosas difusas pelo corpo. O diagnóstico é feito por meio de exame físico, hemocultura, cultura de sítio infectado e análise histopatológica. As medidas de suporte e a antibioticoterapia são a base do tratamento. Este relato descreve o raro acometimento de paciente idoso e evidencia a associação da doença com a imunossupressão. A presença de lesões em mucosa oral é um achado atípico e similar a um dos principais diagnósticos diferenciais da síndrome da pele escaldada estafilocócica: a necrólise epidérmica tóxica. É de suma importância diferenciá-las por meio de análise histopatológica das lesões de pele devido às formas inerentes de tratamento e à gravidade. O relato alerta para a importância do rápido reconhecimento do diagnóstico, a fim de introduzir tratamento adequado precoce, evitando os riscos da terapia inadequada e das complicações naturais da doença. Desta forma, é possível alcançar um desfecho positivo em uma doença de baixa prevalência e alta mortalidade no adulto.(AU)

The Staphylococcal Scalded Skin Syndrome is a rare exfoliative dermatitis in adults, affecting about 0.09 to 0.56 per million inhabitants. It is caused by exotoxins produced by Staphylococcus aureus and is manifested by the formation of diffuse bullous body lesions. The diagnosis is made through physical examination, blood culture, infected site culture, and histopathology. Supportive measures and antibiotics are the mainstay of the treatment. This report describes the rare involvement of an elderly patient, and highlights the association of the disease with immunosuppression. The presence of lesions in the oral mucosa is an atypical finding, being similar to one of the main differential diagnosis of the Staphylococcal Scalded Skin Syndrome: the Toxic Epidermal Necrolysis. It is very important to differentiate them through histopathology of the skin lesions due to the peculiar forms of treatment and the severity of the diseases. The report highlights the importance of rapid recognition of diagnosis in order to introduce early appropriate treatment, avoiding the risks of inappropriate therapy, and natural complications of the disease. This way, it is possible to achieve a positive outcome in a disease of low prevalence and high mortality in adults.(AU)

Psoríase artropática eritrodérmica: rápida remissão com secuquinumabe / Erythrodermic psoriatic arthritis: quick remission with secukinumab

Entre 20% e 30% dos pacientes com psoríase vulgar têm formas exuberantes que requerem terapia sistêmica. A imunobiologia representa um ramo promissor da farmacologia moderna e configura um dos grandes avanços da medicina atual. O secuquinumabe é um anticorpo monoclonal de imunoglobulina humana totalmente recombinante, recentemente introduzido no mercado, que atua neutralizando seletivamente a interleucina17-A e promove excelentes resultados no tratamento de casos moderados a graves de psoríase. Apresentamos uma paciente de 66 anos internada por psoríase artropática eritrodérmica e tratada com secuquinumabe sob regime de monoterapia. Houve melhora importante das lesões cutâneas após 6 semanas e resolução completa do quadro, inclusive da artrite, após 10 semanas de tratamento. (AU)

Between 20% and 30% of patients with psoriasis have exuberante forms that require systemic therapy. Immunobiology represents a promising branch of modern pharmacology and is one of the great advances of current medicine. Secukinumab is a recently released recombinant human immunoglobulin monoclonal antibody that selectively neutralizes interleukin17A and promotes excelente results in the treatment of moderate to severe cases of psoriasis. We present a 66 year old patient hospitalized for erythrodermic arthropathic psoriasis and treated by a monotherapy regimen with secukinumab. There was significant mprovement of skin lesions after 6 weeks and complete resolution of the condition, including the arthritis, after 10 weeks of treatment. (AU)

A case series of dermatological emergencies – Erythroderma

Erythroderma can be life-threatening, primarily because of its metabolic burden and complications. It is mandatory to establish its etiopathology in order to facilitate precise and definitive management. This disorder may be the morphologic presentation of a variety of cutaneous and systemic diseases. Detailed history and thorough work-up is therefore essential. Management of erythroderma involves multi-disciplines with progress monitoring especially on signs and symptoms suggestive of acute skin failure induced complications. Early diagnosis and referral of erythroderma to centres with dermatological services is crucial and will directly affect the outcome of the patients.