Resumo O sarcoma epitelióide (SE) é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como “tipo proximal”. São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com amputação abdominoperineal do reto por videolaparoscopia associada a hemipelvectomia parcial esquerda incluindo o membro inferior esquerdo (MIE). O paciente apresentou boa evolução pós operatória e encontra-se em segmento ambulatorial. O anatomopatológico evidenciou margens cirúrgicas livres.O SE proximal é um tipo raro de sarcoma de partes moles, cujo tratamento curativo é desafiador, em virtude dos altos índices de recidiva local e metástases à distância.

Abstract The epithelioid sarcoma (ES) is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the “proximal-type” in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with “proximal-type” ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The “proximal-type” ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task.

Biologic drugs represent a substantial progress in the treatment of chronic inflammatory immunologic diseases. However, its crescent use has revealed seldom reported or unknown adverse reactions, mainly associated with anti-tumor necrosis factor (anti-TNF). Psoriasiform cutaneous reactions and few cases of alopecia can occur in some patients while taking these drugs. Two cases of alopecia were reported after anti-TNF therapy. Both also developed psoriasiform lesions on the body. This is the second report about a new entity described as 'anti-TNF therapy-related alopecia', which combines clinical and histopathological features of both alopecia areata and psoriatic alopecia. The recognition of these effects by specialists is essential for the proper management and guidance of these patients.

FUNDAMENTOS: A dermatoscopia é método importante de diagnóstico de doenças melanocíticas benignas e malignas. Recentemente, o uso desse método tem demonstrado grande ajuda também no diagnóstico e acompanhamento das alopecias. OBJETIVO: Descrever e demonstrar os achados dermatoscópicos de pacientes com quadros clínicos e histopatológicos de alopecia cicatricial. MÉTODOS: Estudo transversal descritivo em que foram selecionados, pelos achados clínicos e histopatológicos do couro cabeludo, 14 pacientes com alopecia cicatricial, sendo quatro casos de líquen plano pilar clássico, cinco casos de alopecia fibrosante frontal e cinco com lúpus eritematoso discoide. Os pacientes foram avaliados com videodermatoscópio e dermatoscópio manual por três examinadores diferentes, de forma independente. Os aumentos variaram de 10 a 70 vezes. RESULTADOS: Foram achados predominantes no lúpus eritematoso discoide placas brancas, capilares arboriformes, tampões ceratósicos e áreas com diminuição dos óstios foliculares; no líquen plano pilar clássico, escamas perifoliculares, pontos brancos e diminuição de óstios foliculares; na alopecia fibrosante frontal, diminuição de óstios foliculares, escama e eritema perifolicular, além de capilares arboriformes. As estruturas azul-acinzentadas, demonstradas neste estudo, não foram descritas na literatura. CONCLUSÕES: O uso da dermatoscopia na avaliação clínica das alopecias ajudou a estabelecer elementos semióticos, melhorou a capacidade de diagnóstico em relação à simples inspeção e revelou novas características das alopecias cicatriciais.

BACKGROUND: Dermoscopy is an important tool for the diagnosis of benign and malignant melanocytic diseases. Recently, this method has also been found to be extremely useful in the diagnosis and follow-up of alopecias. OBJECTIVE: The objective of this study was to describe dermoscopic findings in patients with clinical and histopathological characteristics of cicatricial alopecia. METHODS: A descriptive cross-sectional study was conducted in which 14 patients with cicatricial alopecia were selected based on clinical and histopathological evaluation of the scalp. The underlying cause was classic lichen planopilaris in four cases, frontal fibrosing alopecia in five and discoid lupus erythematosus in the remaining five. The patients were evaluated using videodermoscopy and conventional dermoscopy (with a handheld dermoscope), performed independently by three different examiners. Magnification ranged from 10x to 70x. RESULTS: Principal findings in cases of discoid lupus erythematosus were: white patches, branching capillaries, keratin plugs and areas of reduced follicular ostia; in classic lichen planopilaris: perifollicular scales, white dots and reduced follicular ostia; and in frontal fibrosing alopecia: reduced follicular ostia, perifollicular scales, perifollicular erythema and branching capillaries. The blue-grey dots described in this paper were a novel feature in scalp dermoscopy. CONCLUSIONS: The use of dermoscopy for the clinical evaluation of the scalp in cases of cicatricial alopecia improves diagnostic capacity beyond simple clinical inspection and reveals novel features of the disease.

A psoríase possui diversas formas de apresentações clínicas. Lesões hiperceratósicas podem ser observadas e verificam-se na literatura diversas denominações para essas lesões, como elefanthine, rupióide, ostrácea e pseudocórnea. É interessante notar que duas dessas denominações se remetem à semelhança com conchas encontradas nos estudos de conquiliologia: rupióide e ostrácea. Os autores comparam os aspectos morfológicos das variantes da psoríase hiperceratósica com as conchas de diversas espécies de moluscos.

Psoriasis is a skin disorder with several clinical expressions. Hyperkeratotic lesions may be observed, and have been named rupioid, elephantine, ostraceous and pseudohorny in the literature. It is worth noting that two of such denominations refer to the similarity they bear with shells found by the study of conchology, namely rupioid and ostraceous. The authors compare the morphological aspects of hyperkeratotic psoriasis variants with the shells of different mollusk species.