High-risk mucosal HPV types associated with squamous cell carcinoma on the nose tip in an immunocompetent young man

Abstract: The oncogenic role of high-risk HPV in anogenital, head and neck, and cervical cancer is well recognized, but not in skin cancer in the general population. Some authors have demonstrated their appearance mainly on the hands and feet, particularly in the area of the nail bed, which could be due to contamination with HPV types from anogenital regions. Here, we describe a case of genital HPV associated with SCC on the nose tip in an immunocompetent young man, which was confirmed by histopathological findings and in situ hybridization. The importance of this report is to highlight the potential role of HPV in the etiology of skin cancer in an immunocompetent individual.

Melanoma: tumor microenvironment and new treatments

Abstract: In the recent past years, many discoveries in the tumor microenvironment have led to changes in the management of melanoma and it is rising up hopes, specially, to those in advanced stages. FDA approved seven new drugs from 2011 to 2014. They are: Vemurafenib, Dabrafenib and Trametinib, kinases inhibitors used for patients that have BRAFV600E mutation; Ipilimumab (anti-CTLA4), Pembrolizumab (anti-PD-1) and Nivolumab (anti-PD-1), monoclonal antibodies that stimulate the immune system; and Peginterferon alfa-2b, an anti-proliferative cytokine used as adjuvant therapy. In this article, we will review the molecular bases for these new metastatic melanoma therapeutic agents cited above and also analyze new molecular discoveries in melanoma study, as Cancer-Testis antigens (CT). They are capable of induce humoral and cellular immune responses in cancer patients and because of this immunogenicity and their restrict expression in normal tissues, they are considered an ideal candidate for vaccine development against cancer. Among CT antigens, NY-ESO-1 is the best characterized in terms of expression patterns and immunogenicity. It is expressed in 20-40% of all melanomas, more in metastatic lesions than in primary ones, and it is very heterogeneous inter and intratumoral. Breslow index is associate with NY-ESO-1 expression in primary cutaneous melanomas, but its relation to patient survival remains controversial.

Angiosarcoma in HIV-negative patients is not associated with HHV-8

Abstract: BACKGROUND: Angiosarcoma is an aggressive, malignant neoplasm of vascular or lymphatic origin. Herpes virus 8 (HHV-8) is a member of the herpes family with a tropism for endothelial cells and it has been proven to induce vascular neoplasms, such as Kaposi's sarcoma. The role of HHV-8 in the pathogenesis of angiosarcoma has not been well defined. OBJECTIVE: To investigate the relationship between the presence of HHV-8 and angiosarcoma. METHODS: In this study, the team investigated the relationship between the presence of HHV-8, as determined by polymerase chain reaction, and angiosarcoma, using samples from patients with epidemic Kaposi's sarcoma as controls. RESULTS: While all control cases with epidemic Kaposi's sarcoma were positive for HHV-8, none of the angiosarcoma cases was. CONCLUSION: These findings support most previous studies that found no association between HHV-8 and angiosarcoma.

Inflammasomes and dermatology

Abstract: Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies. Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA). Concepts from other pathologies have also been reviewed in recent years, such as psoriasis, after the recognition of a combined contribution of innate and adaptive immunity in its pathogenesis. Inflammasomes are also involved in the response to various infections, malignancies, such as melanoma, autoimmune diseases, including vitiligo and lupus erythematosus, atopic and contact dermatitis, acne, hidradenitis suppurativa, among others. Inhibition of the inflammasome pathway may be a target for future therapies, as already occurs in the handling of CAPS, through the introduction of IL-1 inhibitors. This study presents a literature review focusing on the participation of inflammasomes in skin diseases.

Profile of patients admitted to a triage dermatology clinic at a tertiary hospital in São Paulo, Brazil

Abstract: Background: Knowledge of epidemiological data on skin diseases is important in planning preventive strategies in healthcare services. Objective: To assess data from patients admitted to a triage dermatology clinic. Methods: A retrospective study was performed of patients admitted over a one-year period to the Triage Dermatology Clinic at the Hospital das Clínicas of the University of São Paulo Medical School. Data were obtained from record books. The variables analyzed were: patient age, gender, dermatologic disease (initial diagnosis), origin (from where the patient was referred) and destination (where the patient was referred to). Results: A total of 16,399 patients and 17,454 diseases were identified for analysis. The most frequent skin disorders were eczema (18%), cutaneous infections (13.1%), erythematous squamous diseases (6.8%) and malignant cutaneous neoplasms (6.1%). Atopic dermatitis was the most common disease in children. Acne was more common among children and adults, as were viral warts. Basal cell carcinoma and squamous cell carcinoma were more common in the elderly. Contact dermatitis and acne predominated in women. The most frequent origins were: the primary/secondary health system (26.6%), other outpatient specialties (25.5%), emergency care (14.9%); while the destinations were: discharged (27.5%), follow-up in our Dermatology Division (24.1%), return (14.1%) and the primary/secondary health system (20.7%). Conclusion: Understanding the incidence of skin diseases is fundamental in making decisions regarding resource allocation for clinical care and research. Thus, we believe our findings can contribute to improving public health policies.

Phaeohyphomycosis in renal transplantation: report of two cases

Abstract Phaeohyphomycosis is an infection caused by a filamentous fungus that contains pigment melanin in its cell wall. We report two cases caused by Exophiala sp. emphasizing the clinical variability of the disease, as well as diagnostic and therapeutic difficulties of this opportunistic infection in immunosuppressed patients (kidney transplant).

Cutaneous field cancerization: clinical, histopathological and therapeutic aspects / Campo de cancerizacao cutaneo: implicacoes clinicas, histopatologicas e terapeuticas

The concept of "field cancerization" was first introduced by Slaughter in 1953 when studying the presence of histologically abnormal tissue surrounding oral squamous cell carcinoma. It was proposed to explain the development of multiple primary tumors and locally recurrent cancer. Organ systems in which field cancerization has been described since then are: head and neck (oral cavity, oropharynx, and larynx), lung, vulva, esophagus, cervix, breast, skin, colon, and bladder. Recent molecular studies support the carcinogenesis model in which the development of a field with genetically altered cells plays a central role. An important clinical implication is that fields often remain after the surgery for the primary tumor and may lead to new cancers, designated presently as "a second primary tumor" or "local recurrence," depending on the exact site and time interval. In conclusion, the development of an expanding pre-neoplastic field appears to be a critical step in epithelial carcinogenesis with important clinical consequences. Diagnosis and treatment of epithelial cancers should not only be focused on the tumor but also on the field from which it developed. The most important etiopathogenetic, clinical, histopathological and therapeutic aspects of field cancerization are reviewed in this article.

O conceito de campo de cancerização foi empregado, pela primeira vez, por Slaughter em 1953, estudando o tecido alterado peri-tumoral de carcinoma espinocelular, através da histopatologia. O conceito foi proposto para explicar o desenvolvimento de múltiplos tumores primários e recorrentes na mesma área onde já havia alterações histopatológicas. Os órgãos que apresentam campo de cancerização, até hoje descritos são: cavidade oral, orofaringe, pulmão, vulva, esôfago, cérvix uterino, pele, mama, cólon e bexiga. Os resultados obtidos a partir da biologia molecular sustentam o modelo de carcinogênese, onde um campo com células geneticamente alteradas têm papel fundamental. Uma importante implicação clínica é o fato de que campos com células mutadas geralmente permanecem após a cirurgia para a remoção de um tumor primário, podendo originar novos tumores, designados como segundo tumor primário ou recorrência local, dependendo do local exato e do intervalo entre a cirurgia e a detecção deste novo tumor. O desenvolvimento de campos com células mutadas é considerado uma etapa crítica na carcinogênese, com importante repercussão clínica. Assim, o diagnóstico e tratamento das lesões malignas epiteliais deve abordar não somente o tumor isolado mas sim, todo o campo onde se desenvolveu. Neste artigo, revisamos aspectos importantes da etiopatogenia, clínica, histopatologia e da terapêutica do campo de cancerização.

Prevalence of depression and anxiety disorders in hospitalized patients at the dermatology clinical ward of a university hospital / Prevalência de depressão e ansiedade em pacientes hospitalizados na enfermaria da clínica de dermatologia de um hospital universitário

BACKGROUND: The objective of this study was to estimate the prevalence of depression and anxiety disorders in hospitalized patients at the dermatology ward at a university hospital in São Paulo, Brazil. OBJECTIVE: To assess the prevalence of mood and anxiety disorders in hospitalized patients at the dermatology ward at a university hospital in São Paulo. METHOD: A total of 75 patients, men and women, aged between 18 and 76 years, took part in the research. The study employed a descriptive, cross sectional and correlational method. The data was collected by means of a social demographic questionnaire and the PRIME-MD. RESULTS: It was found that 45.3 percent of the subjects presented with depressive symptoms, and 52 percent presented with symptoms of anxiety and that this survey showed moderate and high significant correlations (p<0,01; r= 0,616) for depression and anxiety. CONCLUSION: These facts could evidence the relationship between physical and psyche, just as the literature presents.

FUNDAMENTOS: O presente estudo teve como objetivo verificar a freqüência de depressão e ansiedade em pacientes internados na Divisão da Clínica de Dermatologia de um hospital universitário de São Paulo. OBJETIVO: Avaliar a prevalência de depressão e ansiedade em pacientes hospitalizados na enfermaria da clínica de dermatologia de um hospital universitário em São Paulo. MÉTODO: Participaram da pesquisa 75 sujeitos, homens e mulheres, entre 18 e 76 anos. O delineamento do estudo foi transversal e descritivo. Os instrumentos utilizados foram Entrevista Sócio Demográfica e PRIME-MD. RESULTADOS: Identificou-se a presença de depressão em 45,3% e de ansiedade em 52% dos pacientes avaliados. CONCLUSÃO: Verificou-se correlação moderada e altamente significativa (p<0,01; r =0,616) para os índices de depressão e ansiedade, que pode evidenciar a relação entre adoecimento físico e psíquico muito encontrada na literatura.

Perfil epidemiológico e histopatológico de 642 casos de carcinoma basocelular de cabeça e pescoço operados em uma instituição terciária / Epidemiological and histopathological profile of 642 cases of basal cell carcinoma of head and neck surgery in a tertiary institution

Introdução: Carcinoma basocelular (CBC) é o câncer de pelemais frequente. A maioria dos CBCs tem comportamentoindolente, com cura em 95% dos casos após tratamentolocal. Uma pequena parcela corresponde a lesões de altamorbidade tratadas pela Cirurgia de Cabeça e Pescoço (CCP).A caracterização desses casos é pouco encontrada. Objetivo:Avaliar o perfil epidemiológico dos pacientes operados ecaracterísticas anatomopatológicas de CBCs em uma instituiçãoterciária. Método: Prontuários de 642 pacientes operados porCBC foram analisados, sendo comparados os dados de 322casos tratados pela CCP (grupo CCP) com 320 pela Dermatologia(grupo Derma). Sexo, idade, localização do tumor primário,zona de acometimento, tamanho, subtipo histológico e invasõesperineural e angiolinfática foram avaliados. Resultados: 51,8%dos pacientes do grupo CCP e 45,9% do grupo Derma eramhomens (p=0,13). A idade média dos grupos CCP e Dermaforam de 58,8±11,4 e 76,1±10,1 anos (p<0,01). A zona H foisede de 78,9% dos tumores do grupo CCP e 71,9% do grupoDerma (p=0,04). A mediana do tamanho tumoral foi de 2,5cm(1,45-4,0) no grupo CCP e 0,6cm (0,4-1,0) no grupo Derma(p<0,01). Os subtipos esclerodermiforme e adenoide forammais frequentes no grupo CCP (p<0,001), enquanto nodular eesclerosante foram no grupo Derma(p<0,001). Invasão perineuralfoi observada em 22,5% no grupo CCP e 13,2% no grupo Derma(p<0,01). Conclusão: A caracterização dos casos tratados pelaCCP permite maior conhecimento sobre lesões agressivas epossivelmente a identificação precoce dos casos de maior risco.

Genomic instability in human actinic keratosis and squamous cell carcinoma

OBJECTIVE: To compare the repetitive DNA patterns of human actinic keratoses and squamous cell carcinomas to determine the genetic alterations that are associated with malignant transformation. INTRODUCTION: Cancer cells are prone to genomic instability, which is often due to DNA polymerase slippage during the replication of repetitive DNA and to mutations in the DNA repair genes. The progression of benign actinic keratoses to malignant squamous cell carcinomas has been proposed by several authors. MATERIAL AND METHODS: Eight actinic keratoses and 24 squamous cell carcinomas (SCC), which were pair-matched to adjacent skin tissues and/or leucocytes, were studied. The presence of microsatellite instability (MSI) and the loss of heterozygosity (LOH) in chromosomes 6 and 9 were investigated using nine PCR primer pairs. Random Amplified Polymorphic DNA patterns were also evaluated using eight primers. RESULTS: MSI was detected in two (D6S251, D9S50) of the eight actinic keratosis patients. Among the 8 patients who had squamous cell carcinoma-I and provided informative results, a single patient exhibited two LOH (D6S251, D9S287) and two instances of MSI (D9S180, D9S280). Two LOH and one example of MSI (D6S251) were detected in three out of the 10 patients with squamous cell carcinoma-II. Among the four patients with squamous cell carcinoma-III, one patient displayed three MSIs (D6S251, D6S252, and D9S180) and another patient exhibited an MSI (D9S280). The altered random amplified polymorphic DNA ranged from 70 percent actinic keratoses, 76 percent squamous cell carcinoma-I, and 90 percent squamous cell carcinoma-II, to 100 percent squamous cell carcinoma-III. DISCUSSION: The increased levels of alterations in the microsatellites, particularly in D6S251, and the random amplified polymorphic DNA fingerprints were statistically significant in squamous cell carcinomas, compared with actinic keratoses. CONCLUSION: The overall alterations that were observed in the repetitive DNA of actinic keratoses and squamous cell carcinomas indicate the presence of a spectrum of malignant progression.

Inpatient dermatological consultations in a university hospital

INTRODUCTION: Dermatology is primarily an outpatient specialty, but it also plays an important role in the care of inpatients. METHODS: We conducted a prospective study that recorded data from inpatient dermatology consultation request forms over a period of four months. The study evaluated 313 requests that led to 566 visits, 86 biopsies, 35 laboratory exams, 41 direct microscopic studies, 18 direct immunofluorescence analyses, 14 skin cultures and a few other exams. RESULTS: The most frequent requesting service was internal medicine (24 percent), followed by neurology (12 percent), cardiology (11 percent), infectious diseases and pediatrics (8 percent each) and psychiatry and general surgery (6 percent each). The most frequent diagnostic groups were infectious diseases (25 percent, divided into fungal infections (13 percent), bacterial infections (7 percent) and viral infections (5 percent)), eczemas (15 percent) and drug reactions (14 percent). To our knowledge, this is the first study to attempt to evaluate the impact of the consultations by asking multiple-choice questions that were analyzed by the authors. In 31 percent of the cases, the consultation was considered extremely relevant because it aided in managing the disease that led to admission or treated a potentially severe dermatological disease. In 58 percent of the cases, the consultation was considered important because it facilitated diagnosis and/or treatment of a dermatological disease that was unrelated to the reason for admission.

Treatment of cutaneous tumors with topical 5 percent imiquimod cream

INTRODUCTION: There are various approaches to the treatment of cutaneous tumors; one of them is treatment with imiquimod, a synthetic toll-like receptor agonist with a low molecular weight that offers a topical, noninvasive, and non-surgical therapeutic option. The main objective of our study was to provide data on 89 patients who used a 5 percent imiquimod cream for the treatment of cutaneous tumors at the Cutaneous Oncology Group of the Dermatology Department of Hospital das Clinicas from 2003 to 2008. MATERIALS AND METHODS: Here, we present our experience in the treatment of 123 cutaneous tumors of various types, including basal cell carcinoma (BCC), squamous cell carcinoma (SCC), Bowen's disease, erythroplasia of Queyrat, Paget's disease, and trichoepithelioma, with 5 percent imiquimod cream from 2003 to 2008 in the Cutaneous Oncology Group of the Dermatology Department of Hospital das Clinicas. Patients were divided into two separate groups according to their diagnosis and comorbidities; these comorbidities included epidermodysplasia verruciformis, xeroderma pigmentosum, albinism, basal cell nevus syndrome, Brooke-Spiegler syndrome, HIV, chronic lymphocytic leukemia, B-cell lymphoma, and kidney transplantation. Treatment duration, response to imiquimod, follow-up, recurrence, and local and systemic reactions associated with use of the drug were analyzed. Epidemiological data were obtained and cure rates were calculated. RESULTS: The ratio of women to men was 1.28:1, and the mean age was 63.1 years. Tumors were located mainly on the face, back, trunk, and legs. For patients with comorbidities, the overall cure rate was 38 percent. These specific patients demonstrated cure rates of 83.5 percent for superficial BCC and 50 percent for Bowen's disease. Aggressive BCC and superficial and nodular BCC did not present a good response to treatment. Trichoepitheliomas and nodular BCC showed a partial response, and erythroplasia of Queyrat showed ...

Fatores preditivos do maior número de estádios na cirurgia micrográfica de Mohs para o tratamento do carcinoma espinocelular da cabeça / Predictors of higher number of stages in Mohs micrographic surgery for the treatment of squamous cell carcinoma of the head

FUNDAMENTOS: Os carcinomas espinocelulares da pele da cabeça têm como opção terapêutica mais segura a cirurgia micrográfica de Mohs, que apresenta os menores índices de recidiva e a máxima preservação tecidual. Características dos carcinomas espinocelulares podem estar relacionadas a maior número de estádios cirúrgicos. OBJETIVO: Definir características dos carcinomas espinocelulares que sejam preditoras de maior número de estádios na cirurgia de Mohs. MÉTODOS: Análise retrospectiva de 51 carcinomas espinocelulares da cabeça tratados pela cirurgia de Mohs para determinar fatores de risco de maior número de estádios. Foram analisados limites clínicos, morfologia, recidiva, histologia e tamanho, relacionando-os ao número de estádios cirúrgicos. A análise estatística foi realizada pelo teste exato de Fisher e regressão logística multivariada. RESULTADOS: Os carcinomas recidivados tiveram tendência a maior número de estádios (p=0,081). Os tumores com limites imprecisos apresentaram três vezes mais possibilidades de maior número de fases na análise da razão de chances. Esse achado foi compatível com dados da literatura, apesar de não ter sido estatisticamente significante. CONCLUSÃO: Características pré-operatórias dos carcinomas espinocelulares, como recidiva e limites imprecisos, apesar de não preditivas, indicaram tendência a maior número de estádios na cirurgia micrográfica de Mohs.

BACKGROUND: Squamous cell carcinomas of the skin of the head are better treated with Mohs micrographic surgery which has the lowest recurrence rates and allows spare normal tissue. There are some characteristics of squamous cell carcinoma that can be related to a higher number of surgical stages. OBJECTIVE: To study characteristic of head squamous cell carcinoma that predicts a higher number of Mohs surgical stages. METHODS: A retrospective analysis of 51 squamous cell carcinomas of the head treated with Mohs surgery was performed to determine risk factors for a higher number of surgical stages. The characteristics analyzed were clinical limits, morphology, recurrence, histological differentiation and size and compared to the number of surgical stages. The analysis was performed by Fisher’s exact test and multivariate logistic regression. RESULTS: The recurrent squamous cell carcinomas showed a tendency for a higher number of stages (p=0,081). The Odds Ratio for a higher number of Mohs stages was three for inaccurate limits; although not statistically significant,it corroborates clinical and previous publication. CONCLUSION: Clinical characteristics of squamous cell carcinoma as recurrence and inaccurate limits would not predict, but could indicate tendency of a higher number of Mohs micrographic surgery stages.

Genética molecular aplicada ao câncer cutâneo não melanoma / Molecular genetics of non-melanoma skin cancer

Os cânceres cutâneos não melanoma são as neoplasias malignas mais comuns em humanos. O carcinoma basocelular e o carcinoma espinocelular representam cerca de 95 por cento dos cânceres cutâneos não melanoma, o que os torna um crescente problema para a saúde pública mundial devido a suas prevalências cada vez maiores. As alterações genéticas que ocorrem no desenvolvimento dessas malignidades cutâneas são apenas parcialmente compreendidas, havendo muito interesse no conhecimento e determinação das bases genéticas dos cânceres cutâneos não melanoma que expliquem seus fenótipos, comportamentos biológicos e potenciais metastáticos distintos. Apresenta-se uma revisão atualizada da genética molecular aplicada aos cânceres cutâneos não melanoma, em especial ao carcinoma basocelular e carcinoma espinocelular, enfatizando os mais freqüentes genes e os principais mecanismos de instabilidade genômica envolvidos no desenvolvimento dessas malignidades cutâneas.

Non-melanoma skin cancers are the most common malignant neoplasms in humans. About 95 percent of all non-melanoma skin cancers are represented by basal cell carcinoma and squamous cell carcinoma. Their prevalences are still increasing worldwide, representing an important public health problem. The genetic alterations underlying basal cell carcinoma and squamous cell carcinoma development are only partly understood. Much interest lies in determining the genetic basis of non-melanoma skin cancers, to explain their distinctive phenotypes, biological behaviors and metastatic potential. We present here a molecular genetic update, focusing on the most frequent genes and genomic instability involved in the development and progression of non-melanoma skin cancers.

Angioma em tufos: relato de um caso tratado com laser corante pulsado / Tufted angioma: a case report treated with pulsed-dye laser

O angioma em tufos é quadro raro, benigno, de proliferação cutânea angiomatosa. Acomete mais crianças e clinicamente caracteriza-se por lesões em placas, vermelho-violáceas, dolorosas e mais freqüentes no tronco. Quando não regride espontaneamente, os tratamentos propostos (cirurgia convencional, corticoterapia, interferon e laser) alcançam pouco sucesso. Descreve-se o caso de uma menina de 11 anos, com angioma em tufos no tronco, tratada com laser corante pulsado que mostrou alívio importante da dor local e redução discreta do tamanho da lesão. A raridade de casos relatados no Brasil e a tentativa terapêutica com laser corante pulsado motivaram a divulgação do caso.

Tufted angioma is a rare, benign, cutaneous angiomatous proliferation. It is more common in children and is clinically characterized by red-purple painfull plaques, more common in trunk. When spontaneous regression does not occur, proposed treatments (conventional surgery, steroid therapy, interferon and laser) achieve little success. We describe a case of a 11-year-old girl with a tufted angioma located in the trunk treated with pulsed-dye laser, showing important relief of local pain, and small reduction in lesion size. We have been motivated to describe this case due to the small number of such reports in Brazil and by the attempt to treat it with pulsed-dye laser.

Processos linfoproliferativos da pele: parte 2 - linfomas cutâneos de células T e de células NK / Processos linfoproliferativos da pele: part 2 - cutaneous T-cell and NK-cell lymphomas

Os linfomas cutâneos de células T/NK constituem um grupo de doenças linfoproliferativas extranodais atualmente classificadas e subdivididas de acordo com o comportamento clínico segundo consenso da Organização Mundial de Saúde e da Organização Européia para Pesquisa e Tratamento do Câncer. Os linfomas cutâneos de células T/NK de comportamento clínico indolente compreendem a micose fungóide clássica, a micose fungóide foliculotrópica, a reticulose pagetóide, a cútis laxa granulomatosa, o linfoma cutâneo primário de grande célula anaplásica, a papulose linfomatóide, o linfoma subcutâneo de célula T paniculite-símile e o linfoma cutâneo primário de pequena e média célula T CD4+ pleomórfica. Os linfomas cutâneos de células T/NK de comportamento agressivo incluem a síndrome de Sézary, o linfoma extranodal de célula T/NK, tipo nasal, o linfoma cutâneo primário agressivo de célula T CD8+ epidermotrópica, o linfoma cutâneo de célula T gd e o linfoma cutâneo primário de célula T periférica, não especificado. O linfoma-leucemia de células T do adulto e a neoplasia hematodémica CD4+CD56+, embora considerados linfomas sistêmicos, são aqui abordados por apresentarem-se inicialmente na pele em significativo número de pacientes. O diagnóstico desses processos é realizado pelo exame histopatológico complementado pela análise do fenótipo das células neoplásicas, imprescindível no processo classificatório. O estadiamento para a avaliação da extensão anatômica da doença considera além do envolvimento cutâneo, o estado clínico e histológico dos linfonodos e das vísceras. Avaliação hematológica é fundamental na caracterização da síndrome de Sézary. Os tratamentos preconizados incluem terapêuticas dirigidas exclusivamente à pele, modificadores da resposta biológica e quimioterapia sistêmica.