PURPOSE: This study aimed to evaluate the efficacy of the systemic drugs thalidomide, dapsone, colchicine, and pentoxifylline in the treatment of severe manifestations of RAS. METHODS: An open, 4-year clinical trial was carried out for 21 consecutive patients with severe RAS. Initially, patients were given a 2-week course of prednisone to bring them to a baseline status. Simultaneously, one of the four test drugs was assigned to each patient to be taken for a period of 6 months. During the course of the trial, patients were switched to one of the other three drugs whenever side effects or a lack of satisfactory results occurred, and the 6-month limit of the treatment was then reset. RESULTS: The most efficient and best-tolerated drug was thalidomide, which was administered to a total of eight patients and resulted in complete remission in seven (87.5%). Dapsone was prescribed for a total of nine patients, of whom eight (89%) showed improvement in their symptoms, while five showed complete remission. Colchicine was administered to a total of ten patients, with benefits observed in nine (90%), of whom four showed complete remission. Pentoxyfilline was administered to a total of five patients, with benefits observed in three (60%), of whom one patient showed complete remission. CONCLUSION: The therapeutic methods used in this trial provided significant symptom relief. Patients experienced relapses of the lesions; however, this occurred after withdrawal of their medication during the follow-up period.

Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sarcomas cutâneos primários observando suas características clínicas, etiopatogênicas e histológicas, bem como aspectos do tratamento e evolução. Enfatiza os sarcomas de maior relevância para o dermatologista, como angiossarcoma, dermatofibrossarcoma protuberans, fibroxantoma atípico, leiomiossarcoma, lipossarcoma, tumor maligno de bainha de nervo periférico e sarcoma epitelióide. O sarcoma de Kaposi não é abordado devido a suas características individuais específicas.

Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review.

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 <FONT FACE=Symbol>gd</FONT> 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.

The cutaneous NKT/cell lymphomas are a group of extranodal lymphoproliferative disorders currently classified and subdivided based on their clinical behavior, according to a consensus reached between the World Health Organization and the European Organization for Research and Treatment of Cancer. The cutaneous NKT/cell lymphomas of indolent clinical behavior comprise the classical mycosis fungoides, folliculotropic mycosis fungoides, pagetoid reticulosis, granulomatous slack skin, primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis, subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. The aggressive clinical behavior cutaneous NKT/cell lymphomas include Sézary syndrome, extranodal NK/T-cell lymphoma, nasal type, primary cutaneous aggressive epidermotropic CD8+T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma and primary cutaneous peripheral T-cell lymphoma, unspecified. The adult T-cell leukemia lymphoma and CD4+ CD56+ hematodermic neoplasm are considered systemic lymphomas but are addressed in this article for their initial cutaneous manifestations in a significant number of patients. The diagnosis of these processes is based on histological examination complemented by phenotypic analysis of neoplastic cells, which is essential for classification. The recommended staging is based on type and extension of cutaneous involvement, clinical conditions and histological examination of lymph nodes and organs. Hematological assessment is fundamental to characterize Sézary syndrome. The recommended therapies include exclusively cutaneous treatment, biological response modifiers and systemic chemotherapy.

Os linfomas cutâneos primários de células B pertencem ao grupo das neoplasias malignas originadas de linfócitos B, do tipo não-Hodgkin. A rotina diagnóstica nos processos linfoproliferativos de células B é realizada pela biópsia da pele lesada para a análise histopatológica, imuno-histoquímica e pesquisa do rearranjo gênico. A classificação dos linfomas cutâneos primários vem sendo discutida nos últimos anos; as usualmente utilizadas são as propostas pela World Health Organization - WHO e pela European Organization for Research and Treatment of Cancer - EORTC. A recente classificação consensual proposta por WHO-EORTC deverá substituí-las. Entretanto, apesar dos recentes progressos, ainda existem controvérsias e dificuldades quanto à classificação, ao diagnóstico e ao tratamento dos linfomas cutâneos primários de células B.

Primary cutaneous B-cell lymphomas comprise a group of malignant neoplasms originated from the B-cell lymphoid lineage, of the non-Hodgkin type. The diagnostic routine of cutaneous B-cell lymphoproliferative processes consists of a skin biopsy for histopathological, immunohistochemical and gene rearrangement analysis. The classification of primary cutaneous lymphomas is still controversial. The currently most usual classifications are those proposed by the World Health Organization (WHO) and by the European Organization for Research and Treatment of Cancer (EORTC). The recently proposed WHO-EORTC consensus classification should replace them. However, despite recent progress in this field, there are still controversies and difficulties, mainly regarding the diagnosis and classification of cutaneous B-cell lymphomas.

O eritema necrolítico migratório é uma rara condição cutânea que se apresenta como lesões eritematosas, migratórias, com vesículas e bolhas na superfície, evoluindo para cura com hiperpigmentação. É freqüentemente observado em doentes com tumor de células alfa do pâncreas, ou glucagonoma, e quando associado com intolerância a glicose, anemia, hiperglucagonemia, e perda de peso definem a síndrome do glucagonoma. É descrito o caso de uma paciente do sexo feminino, 52 anos, branca, com eritema necrolítico migratório associado à síndrome do glucagonoma com doença metastática na apresentação, vindo a falecer uma semana após sua admissão. A autópsia mostrou um tumor no corpo do pâncreas diagnosticado como tumor neuroendócrino e confirmado pela imuno-histoquímica. O reconhecimento do eritema necrolítico migratório é de grande importância para a possibilidade de diagnóstico precoce do glucagonoma.

Necrolytic migratory erythema is a rare skin condition that consists of migrating areas of erythema with blisters that heal with hyperpigmentation. It usually occurs in patients with an alpha islet cell tumor of the pancreas-or glucagonoma-and when associated with glucose intolerance, anemia, hyperglucagonemia, and weight loss defines the glucagonoma syndrome. We describe a 52-year-old female patient with necrolytic migratory erythema associated with glucagonoma syndrome who had metastatic disease at presentation and passed away one week after her admission. The autopsy showed a tumor in the body of the pancreas, which was diagnosed as a neuroendocrine tumor and confirmed by immunohistochemistry. The diagnosis of necrolytic migratory erythema is a matter of great importance, since it might be an auxiliary tool for the early detection of glucagonoma.