RESUMO
A escabiose é uma dermatose infecto parasitária mais prevalente no mundo, sobretudo nas regiões tropicais e em países de baixa renda. Surtos são comuns em locais de aglomeração como presídios, escolas e campos de refugiados, e tanto o atraso no diagnóstico como o tratamento inadequado são responsáveis pela propagação da doença. Este trabalho tem por objetivos destacar os principais aspectos da escabiose bem como as apresentações dermatológicas, a fim de auxiliar no diagnóstico e tratamentos precoces, tendo como foco o médico generalista. O estudo foi realizado no Departamento de Clínica de Dermatologia do Hospital das Clínicas da Faculdade de Medicina de São Paulo (HCFMUSP), através de uma revisão de literatura com acesso aos bancos de dados eletrônicos PubMed. A escabiose é causada pelo Sarcoptes scabiei, caracterizada pelo prurido intenso e por lesões cutâneas sugestivas e localizações típicas, mas que podem variar de acordo com a faixa etária ou estados de imunossupressão. É considerada pela Organização Mundial da Saúde uma doença tropical negligenciada, podendo causar grande impacto socioeconômico e, ainda que com menor frequência, levar a complicações, muitas vezes decorrentes de infecções bacterianas secundárias, sobretudo nas formas mais graves da doença (vistas principalmente em pacientes imunossuprimidos). Por esses motivos, o reconhecimento das principais formas de apresentação clínica e sintomas associados são importantes para que o diagnóstico seja estabelecido de forma breve, possibilitando a instituição correta do tratamento e, com isso, cessando o ciclo de transmissão do ácaro.
Assuntos
Sarcoptes scabiei , Ácaros , HipersensibilidadeAssuntos
Transtornos Linfoproliferativos , Pessoas Transgênero , Neoplasias do Colo do Útero , Feminino , Masculino , Humanos , Colo do Útero , PeleRESUMO
Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.
RESUMO
Cutaneous metastases from solid tumors are uncommon events in clinical practice. Most of the time, the patient already has the diagnosis of a malignant neoplasm when the cutaneous metastasis is detected. However, in up to one-third of cases, cutaneous metastasis is identified before the primary tumor. Therefore, its identification may be essential for starting treatment, although it is usually indicative of poor prognosis. The diagnosis will depend on clinical, histopathological, and immunohistochemical analysis. Sometimes the identification of the primary site is difficult; however, a thorough analysis using imaging tests and constant surveillance is important.
Assuntos
Neoplasias Cutâneas , Humanos , Neoplasias Cutâneas/patologiaRESUMO
Sézary syndrome (SS) is a rare and aggressive type of cutaneous T-cell lymphoma, with an abnormal inflammatory response in affected skin. The cytokines IL-1B and IL-18, as key signaling molecules in the immune system, are produced in an inactive form and cleave to the active form by inflammasomes. In this study, we assessed the skin, serum, peripheral mononuclear blood cell (PBMC) and lymph-node samples of SS patients and control groups (healthy donors (HDs) and idiopathic erythroderma (IE) nodes) to investigate the inflammatory markers IL-1B and IL-18 at the protein and transcript expression levels, as potential markers of inflammasome activation. Our findings showed increased IL-1B and decreased IL-18 protein expression in the epidermis of SS patients; however, in the dermis layer, we detected increased IL-18 protein expression. In the lymph nodes of SS patients at advanced stages of the disease (N2/N3), we also detected an enhancement of IL-18 and a downregulation of IL-1B at the protein level. Moreover, the transcriptomic analysis of the SS and IE nodes confirmed the decreased expression of IL1B and NLRP3, whereas the pathway analysis indicated a further downregulation of IL1B-associated genes. Overall, the present findings showed compartmentalized expressions of IL-1B and IL-18 and provided the first evidence of their imbalance in patients with Sézary syndrome.
Assuntos
Interleucina-18 , Síndrome de Sézary , Neoplasias Cutâneas , Humanos , Dermatite Esfoliativa/metabolismo , Inflamassomos/metabolismo , Interleucina-18/genética , Interleucina-18/metabolismo , Leucócitos Mononucleares/metabolismo , Síndrome de Sézary/metabolismo , Pele/metabolismo , Neoplasias Cutâneas/metabolismoRESUMO
Contexto: A mastocitose ocorre devido a uma proliferação neoplásica e clonal de mastócitos que se acumulam em um ou mais sistemas de órgãos. A doença é heterogênea, com manifestações que vão desde lesões cutâneas que podem regredir espontaneamente até neoplasias altamente agressivas associadas à falência de múltiplos órgãos e baixa sobrevida. Não há relatos na literatura de sua associação com líquen plano. Descrição do caso: Relatamos o caso de uma paciente com diagnóstico de mastocitose sistêmica agressiva que apresentou durante o acompanhamento quadro compatível com líquen plano. DiscussaÌo: O diagnóstico da mastocitose sistêmica é baseado em critérios que foram refinados recentemente. O tratamento classicamente envolve bloqueio de mediadores de mastócitos e terapia citorredutora para variantes avançadas da doença. Novas drogas como a midostaurina e o avapritinibe são promissoras. Conclusões: Mesmo não fazendo parte da rotina do dermatologista, a mastocitose sistêmica deve ser uma doença lembrada pelo acometimento da pele e potencial gravidade do quadro.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Mastocitose , Mastocitose Sistêmica , Mutação com Ganho de Função , Líquen Plano , MastócitosRESUMO
Abstract Cutaneous metastases from solid tumors are uncommon events in clinical practice. Most of the time, the patient already has the diagnosis of a malignant neoplasm when the cutaneous metastasis is detected. However, in up to one-third of cases, cutaneous metastasis is identified before the primary tumor. Therefore, its identification may be essential for starting treatment, although it is usually indicative of poor prognosis. The diagnosis will depend on clinical, histopathological, and immunohistochemical analysis. Sometimes the identification of the primary site is difficult; however, a thorough analysis using imaging tests and constant surveillance is important.
RESUMO
ABSTRACT: Epstein-Barr virus-positive mucocutaneous ulcer is a recent and unusual type of lymphoproliferation, mostly associated with various forms of immunosuppression. In most cases, they regress spontaneously, but an increasing number of reports describe a spectral behavior of the lesion, which ranges from a simple ulcer with eosinophilia to aggressive ulcers. In these cases, Epstein-Barr virus-related lymphomas are the main differential diagnosis. We report a unique observation of this rare disease with mandibular involvement. Due to bone erosion, the patient was treated with 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with complete healing of the ulcer on clinical examination and PET-scan control.
Assuntos
Infecções por Vírus Epstein-Barr , Humanos , Protocolos de Quimioterapia Combinada Antineoplásica , Ciclofosfamida , Doxorrubicina , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/diagnóstico , Herpesvirus Humano 4 , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Úlcera/tratamento farmacológico , VincristinaRESUMO
Extranodal natural-killer/T-cell lymphoma (ENKTL) is a rare and aggressive Epstein-Barr virus related mature T-cell and natural-killer malignancy. Although highly prevalent in South America, few studies covering data from this geographic location have been published. Therefore, this study aims to report clinical characteristics, prognostic factors, and outcomes in a multicenter cohort of ENKTL patients from Brazil. This retrospective, observational and multicenter study included 98 ENKTL patients treated during two decades in Brazil. Data were extracted from the T-Cell Brazil Project database. In our cohort, 59/98 patients (60.2%) were male, with a median age of 50 years. Sixty-two patients (63.3%) had B-symptoms, 26/98 (26.5%) had Eastern Cooperative Oncology Group scale ≥ 2; 16/98 (16.3%) presented extranasal disease and 34.7% (34/98) were advanced-stage (Ann Arbor/Cotswolds III/IV). The median follow-up for the whole cohort was 49 months, with an estimated 2-year overall survival (OS) and progression-free survival (PFS) of 51.1% and 17.7%, respectively. In early-stage disease (IE/IIE), the median OS was 21.8 months for patients treated with concurrent radiotherapy plus chemotherapy (CCRT-VIPD [etoposide/vp-16, ifosfamide, cisplatin and dexamethasone), 16.2 months for sequential chemoradiotherapy (SCRT) followed by asparaginase-based regimens, and 56.7 months for SCRT followed by CHOP-like (cyclophosphamide, doxorrubicin, vincristine and prednisone) treatments, p = 0.211. CCRT was associated with higher rates of early-mortality, hematological toxicity, and mucositis. Median OS was 8.2 months for patients with advanced-stage disease receiving regimens containing asparaginase compared to 3.2 months for anthracycline-based therapy, p = 0.851. Chemo-radiotherapy (CRT) regimens demonstrated better OS (p = 0.001) and PFS (p = 0.007) than chemotherapy alone. Multivariate analysis revealed anemia, relapsed/refractory (R/R) disease and radiotherapy omission as poor outcome predictors for OS. Lymphopenia and radiotherapy omission adversely affected PFS. Concerning progression of disease within 24-months (POD-24), clinical stage III/IV was a poor outcome predictor. In this real-life Brazilian cohort, ENKTL presented dismal outcomes. Radiation therapy was an independent factor for increased OS and PFS, but CCRT regimens were associated with higher toxicities. Polychemotherapy based on anti-multi drug resistant agents was not associated with survival benefit in either early or advanced-stage disease in our patient cohort.