Insuficiencia respiratoria en una paciente con síndrome de Sézary / Respiratory failure in a patient with Sézary syndrome

Paciente de 44 años de sexo femenino, sin ninguna enfermedad de base preexistente, con una historia de aproximadamente diez meses de presentar lesiones eritemato-descamativas pruriginosas inicialmente localizadas en extremidades inferiores y que luego se generalizaron en todo el cuerpo, asociándose a la pérdida de peso de aproximadamente 15 kg. El manejo inicial consistió en corticoides tópicos y antihistamínicos orales con poca respuesta clínica. Se inició el estudio por dermatología y se confirmó el diagnóstico inicial de neoplasia cutánea maligna de células T. Luego se realizó el frotis de médula ósea, en el que se identificaron células «cerebriformes¼ que confirmaron el diagnóstico de síndrome de Sézary. La paciente recibió esquema de quimioterapia ciclofosfamida, doxorrubicina, vincristina, etopósido y prednisona. La respuesta inicial fue favorable, con alta hospitalaria y seguimiento en la consulta externa. Transcurridos tres meses de tratamiento, la paciente consultó por episodio febril, tos productiva más distrés respiratorio asociado a estertores basales bilaterales, presentó insuficiencia respiratoria y durante la inducción a la ventilación mecánica sufrió un paro cardiorrespiratorio y falleció

44-year-old female patient, with no preexisting underlying disease, with a history of approximately ten months of presenting pruritic erythematous-desquamative lesions initially localized in the lower extremities and later generalized throughout the body, associated with weight loss of 15 kg. Treatment. Initial management consisted of topical corticosteroids and oral antihistamines with little clinical response. A dermatology wok-up was initiated, and the initial diagnosis of malignant T-cell neoplasm was confirmed. A bone marrow smear was performed, in which "cerebriform" cells were identified, confirming the diagnosis of Sézary syndrome. The patient received cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone chemotherapy. Outcome. The initial response was favorable, with hospital discharge and outpatient follow-up. After three months of treatment, the patient consulted for a febrile episode, productive cough plus respiratory distress associated with bilateral basal rales, presented respiratory failure, and during induction of mechanical ventilation suffered cardiorespiratory arrest and died.

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.

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.

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.

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.

Mutation analysis of interleukin-36RN gene in a child with erythroderma / 中华皮肤科杂志

A 2-year-old male child presented with recurrent diffuse desquamative red macules all over the body,without pustules or ulcers.The patient had repeated fever,which peaked at 39.3 ℃.The patient was diagnosed with erythroderma.Whole genome sequencing showed 2 compound heterozygous mutations (c.28C＞T and c.368C＞T) in the interleukin (IL)-36RN gene.The mutation c.28C＞T was inherited from his father,leading to p.Arg10X and premature termination of amino acid transcription.The mutation c.368C＞T was inherited from his mother,causing p.Thr123 Met.No mutation was found in the IL-1RN gene in the patient.The compound heterozygous mutations c.28C＞T and c.368C＞T may be responsible for erythroderma in this child.

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)