Abstract: Background: Dermoscopy is a noninvasive complementary diagnostic method largely used in dermatology. Feasibility, accuracy, and reproducibility are key elements for a diagnostic method to be useful, hence the importance of the terminology used to describe dermoscopic criteria. Objective: To evaluate the reproducibility of the English descriptive terminology proposed for dermoscopic criteria at the 3rd Consensus Meeting of the International Dermoscopy Society in Brazilian Portuguese. Methods: Nine Brazilian dermatologists independently analyzed the translation of sixty dermoscopic descriptive terms proposed at the 3rd Consensus Conference of the International Society of Dermoscopy. Interobserver agreement index was analyzed using the Fleiss' kappa test. Results: The interobserver agreement of the descriptive terminology in Brazilian Portuguese was considered weak (κ = 0.373; p < 0.05). The interobserver agreement of the descriptive terminology used to describe morphology and arrangement of vascular structures was considered moderate (κ = 0.43; p < 0.05). Study limitations: Our study limitations include the small number of participants and limited regional representation (only 2 out of 5 Brazilian regions were represented). Conclusions: The descriptive English terminology proposed at the 3rd Consensus Conference of the International Dermoscopy Society revealed weak reproducibility and the morphology and arrangement of vascular structures presented moderate reproducibility in Brazilian Portuguese. Despite small regional differences, metaphoric terminology in dermoscopy seems to be the most useful and reproducible system to be adopted in Brazilian Portuguese.

Abstract Pemphigus herpetiformis is an autoimmune bullous disease, that combines clinical features of dermatitis herpetiformis and linear IgA bullous dermatosis and immunological characteristics of pemphigus, which makes this disease peculiar and this diagnosis rarely suspected in the first evaluation of the patient. The reported case is of a patient with clinically bullous disease similar to dermatitis herpetiformis, whose multiple biopsies were inconclusive, and only after direct immunofluorescence with a pemphigus pattern (intraepidermal intercellular pattern) the confirmation of the diagnosis was possible.

Abstract Histiocytoid Sweet's Syndrome is a rare inflammatory disease described in 2005 as a variant of the classical Sweet's Syndrome (SS). Histopathologically, the dermal inflammatory infiltrate is composed mainly of mononuclear cells that have a histiocytic appearance and represent immature myeloid cells. We describe a case of Histiocytoid Sweet's Syndrome in an 18-year-old man. Although this patient had clinical manifestations compatible with SS, the cutaneous lesions consisted of erythematous annular plaques, which are not typical for this entity and have not been described in histiocytic form so far. The histiocytic subtype was confirmed by histopathological analysis that showed positivity for myeloperoxidase in multiple cells with histiocytic appearance.

Abstract The occurrence of multiple primary melanomas in a single individual is rare. Most commonly, malignant melanocytic lesions subsequent to the initial diagnosis of melanoma are secondary cutaneous metastases. We report a patient with gastrointestinal bleeding from gastric metastasis of cutaneous melanoma. During clinical evaluation and staging, we discovered a brain metastasis associated with 3 synchronous primary cutaneous melanomas. We suggest the research on the mutation in the cyclin-dependent kinase inhibitor 2A (CDKN2A) (INK4a) in such cases. We also emphasize the importance of clinical examination and dermoscopy of the entire tegument, even after a malignant melanocytic lesion is identified.

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.

The surgical approach to lentigo maligna is a challenge to dermatologists, given the difficulty of clinical delimitation of borders. We report here a case of a 69-year-old female patient presenting with brownish macules on her face, since 10 years ago, with histopathological diagnosis of lentigo maligna. The surgical management employed was excision of visible borders with the contoured technique and immediate submission of these borders for histopathological analysis before complete excision of the tumor. This technique is a variant of staged excision, with lower rates of recurrence and acceptable aesthetic results.

Mulher de 57 anos, com equimose periorbitária, frouxidão cutânea nas dobras, polineuropatia e síndrome do túnel do carpo bilateral.O exame histopatológico da lesão axilar revelou fragmentação de fibras elásticas, porém a coloração de von Kossa foi negativa;o diagnóstico foi de cútis laxa. Amiloidose sistêmica primária foi confirmada pela presença de material amilóide no exame histopatológico da pálpebra, além de púrpura, polineuropatia, síndrome do túnel do carpo bilateral e gamopatia monoclonal. Foi diagnosticada como portadora de mieloma múltiplo por apresentar 40% de plasmócitos na medula óssea, componente M urinário e anemia. A paciente evoluiu com bolhas de conteúdo citrino, cujo exame histopatológico mostrou mucinose. Os diagnósticos finais foram: amiloidose sistêmica primária, cútis laxa adquirida e mucinose, todos vinculados ao mieloma múltiplo.

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Mastocitose é uma doença rara caracterizada pela proliferação e acúmulo de mastócitos em vários órgãos. A mastocitose cutanea maculopapular divide-se em três subtipos: variante máculo-papular, urticária pigmentosa e telangiectasia macular eruptiva perstans. A dermatoscopia pode ajudar a caracterizar melhor as diferentes formas de mastocitose cutânea. Relatamos um caso de paciente feminina de 55 anos com urticária pigmentosa e sua dermatoscopia.

Mastocytosis is a rare disease characterized by proliferation and accumulation of mast cells in various organs. The maculopapular cutaneus mastocytosis is divided into three subtypes: papular/plaque variant, urticaria pigmentosa and eruptive macular telangiectasia perstans. Dermoscopic may help to better characterize the different forms of cutaneus mastocytosis. We report a 55 year-old female with urticaria pigmentosa and its dermoscopy.

O melanoma acral é o tipo mais prevalente de melanoma na população não caucasiana, sendo a dermatoscopia uma ferramenta útil para um diagnóstico mais precoce e diferenciação com lesões benignas. O padrão dermatoscópico, frequentemente, associado ao melanoma na pele volar é o de cristas paralelas, com 99% de especificidade segundo a literatura. No entanto, também pode ocorrer em diversas lesões acrais benignas, por isso é importante uma boa interpretação deste padrão, associado a história clínica e evolução.

The acral melanoma is the most prevalent type of melanoma in the non-Caucasian population, and dermoscopy is a useful tool for earlier diagnosis and differentiation from benign lesions. The dermoscopic pattern often associated with melanoma on the volar skin is the parallel ridge, with 99% specificity according to the literature. However, this pattern can also occur in several benign acral lesions, so it is important to make a good interpretation of this pattern, along with the clinical history and evolution.

A diferença clínica entre melanoma inicial e tumores benignos pode ser difícil em algumas ocasiões, especialmente na ceratose seborreica pigmentada. A dermatoscopia pode ajudar; porém, os achados nem sempre são conclusivos, de modo que a histopatologia pode ser necessária para o diagnóstico correto. Descrevemos uma lesão melanocítica com aspectos clínicos e dermatoscópicos duvidosos. Nesse caso, a biópsia incisional de área suspeita, guiada pela dermatoscopia, estabeleceu o diagnóstico.

It may be clinically difficult to differentiate early-stage melanoma from benign tumors, specially pigmented seborrheic keratosis. Dermoscopy can help; however, the findings are not always conclusive. Therefore, histopathology may be necessary for a correct diagnosis. We describe a melanocytic lesion with dubious clinic and dermoscopic findings. An incisional biopsy of a suspicious area, guided by dermoscopy, was performed to clarify the findings.

Heteroduplex mobility assay, single-stranded conformation polymorphism and nucleotide sequencing were utilised to genotype human parvovirus B19 samples from Brazil and Paraguay. Ninety-seven serum samples were collected from individuals presenting with abortion or erythema infectiosum, arthropathies, severe anaemia and transient aplastic crisis; two additional skin samples were collected by biopsy. After the procedure, all clinical samples were classified as genotype 1.

A gnatostomíase é parasitose endêmica em alguns países asiáticos, causada pela ingestão da larva do nematódio Gnathostoma sp, que pode ocorrer no consumo de carne crua de peixes de água doce. Atinge vários órgãos, entre eles a pele, manifestando-se frequentemente como lesão subcutânea migratória. Países da América Central e do Sul como México e Peru vêm apresentando número crescente de casos da doença desde 1970. Este trabalho registra o primeiro caso notificado e publicado no Brasil e alerta para o surgimento dessa doença em áreas anteriormente não endêmicas. Relata-se caso de paciente, com história de viagem ao Peru, que evoluiu com quadro cutâneo compatível com gnatostomíase.

Gnathostomiasis is an endemic disease in Asian countries caused by the ingestion of larval stage of Gnathostoma sp roundworms that may be acquired by eating raw fresh-water fish. It can reach many organs and the skin is one of them, characterized by creeping eruptions and/or migrating erythema. Since 1970 the number of patients affected seems to be increasing in Central and South America countries like Mexico and Peru. This is the first notification and publication of gnathostomiasis in Brazil, and it serves as an alert to the possibility of the emergence of this disease in non-endemic areas. We report a case of a man who traveled to Peru and developed cutaneous features accordant with gnathostomiasis.

FUNDAMENTOS - A dermatite esfoliativa/eritrodermia é síndrome rara e grave, de etiologia freqüentemente desconhecida. A hospitalização é necessária para avaliação inicial e tratamento. OBJETIVO - Determinar o perfil clínico, laboratorial e histopatológico dos eritrodérmicos internados no HUCFF-UFRJ. MÉTODOS - No período de 1990 a 2007, 170 casos foram submetidos a hemograma, velocidade de hemossedimentação, bioquímica do sangue, provas de função hepática, proteínas totais e frações, pesquisa de células de Sézary no esfregaço de sangue periférico, radiografia de campos pleuropulmonares e biópsia de pele em três locais. RESULTADOS - Entre 92 homens e 78 mulheres, na faixa de 30 a 80 anos, identificaram-se 99 casos (58,23%) com dermatoses preexistentes; 37 (21,77%) com farmacodermias; 18 (10,58%) com linfomas cutâneos de células T e 16 (9,4%) de causa indeterminada. Calafrios, prurido, linfonodomegalias e edema de membros inferiores destacaram-se dentre sinais e sintomas. O perfil laboratorial consistiu de aumento da VHS, eosinofilia, leucocitose, linfocitose e anemia. No grupo de etiologia indeterminada, dois padrões histológicos emergiram: dermatite psoriasiforme e dermatite inespecífica. CONCLUSÃO - A psoríase foi a causa mais freqüente; três biópsias de pele simultâneas podem aumentar a acurácia do diagnóstico histopatológico.

BACKGROUND - Exfoliative dermatitis (erythroderma) is a rare, severe syndrome, frequently of unknown etiology. The hospitalization is required for the initial evaluation and to establish the treatment. OBJECTIVE - to determine the clinical, laboratorial and histologic profile of erythrodermic inpatients at HUCFF-UFRJ. METHODS - in the period 1990-2007, 170 patients were submitted to complete blood cell count, erythrocyte sedimentation rate, blood urea, glucose and creatinine, liver function tests, proteinogram, circulating Sézary cells count, chest X Rays and skin biopsy of three areas. RESULTS - from 92 male and 78 female aging 30 to 80 years, a preexisting dermatosis was identified in 99 cases (58,23%), reaction to internal drugs in 37 cases (21,77%), T cell cutaneous lymphomas in 18 cases (10,58%); in 16 cases (9,47%), a causative factor could not be disclosed. Chills, pruritus, lymphadenopathy, lower limbs edema were the most freqüent signs/symptoms. Elevated erythrocyte sedimentation rate, eosinophilia, leukocytosis, peripheral blood lymphocytosis and anemia were the most common laboratory findings. Among the idiopathic cases, two histological patterns were noticed: psoriasiform and inespecific dermatitis. CONCLUSION - the main cause of erythroderma was psoriasis; three simultaneous skin biopsies can enhance the accuracy of the histopathologic diagnosis.

Sete casos de hanseníase multibacilar (MB) e dois casos com suspeição de hanseníase atendidos em situações distintas do atendimento clínico-dermatológico na Universidade Federal do Rio de Janeiro são descritos. Todos apresentaram dificuldades no diagnóstico visto que não tinham sinais e sintomas cardinais da hanseníase. Um teste sorológico utilizado como ferramenta auxiliar foi útil no processo de diagnóstico ou exclusão de cada caso e facilitou as discussões acadêmicas na hora do exame clínico. A sorologia e baciloscopia de linfa são consideradas como os únicos instrumentos rápidos e de baixo custo para a confirmação de casos MB atípicos, e as vantagens e desvantagens de cada exame são discutidas. Ambos os testes complementam o processo diagnóstico e classificação dos casos para fins terapêuticos. A vantagem da baciloscopia está na sua capacidade de confirmação do diagnóstico. As vantagens da sorologia são: (a) sua aplicabilidade para uso direto por profissionais de saúde no momento da consulta, visto que os resultados são imediatos, (b) a possibilidade da participação dos pacientes no processo, e (c) oferece uma oportunidade para melhor ensino da patogênese da hanseníase.

Seven multibacillary leprosy and two suspected cases assisted in different situations during clinical care activities at the university in Rio de Janeiro city are described. All cases presented some difficulties for diagnosis, since they evolved with few or no cardinal signs or symptoms of leprosy. A serological test used as an auxiliary tool was helpful in the diagnosis or exclusion procedure of each case, facilitating academic discussions at the time of case examination. Considering serology and bacilloscopy (skin smear) as the only rapid and relatively cheap available tests for confirmation of atypical MB leprosy, the advantages and disadvantages of their use were discussed. Both tests support the diagnostic procedure and the classification of cases for treatment purposes. The advantage of bacilloscopy is its capacity for diagnosis confirmation. The advantages of serology are: (a) its applicability for direct use by health workers, providing immediate results; (b) the potential for patient participation in the process; and (c) it provides a learning opportunity, allowing for improved teaching of leprosy pathogenesis.

Relato de 12 casos de úlcera de Marjolin observados de 1990 a 2003 no HUCFF-UFRJ. Cinco pacientes do sexo feminino e sete do masculino, com idade variando de 38 a 86 anos. Tempo de evolução de 10 a 50 anos, da cicatriz até surgimento do carcinoma espinocelular. Ressecção da lesão em dois casos e amputação terapêutica em oito. Em um caso realizada exérese paliativa da lesão por apresentar metástase para coluna sacrococcígea. Um caso de impossibilidade terapêutica. Concluiu-se que a biópsia é essencial em úlceras crônicas, para diferenciar carcinoma espinocelular de hiperplasia pseudoepiteliomatosa.

The authors report twelve cases of Marjolin's ulcer that have been diagnosed between 1990 and 2003 at HUCFF-UFRJ. Five females and seven males, aged 38 to 86 years old, formed the group. Evolution time from scar to squamous cell carcinoma onset ranged from 10 to 50 years. Amputation was performed in eight cases and in one of them radiation therapy was associated. Two patients underwent local excision. One patient who already had spinal metastasis was submitted to palliative local excision. Finally, one case had no therapeutic possibilities. Early biopsy was concluded to be essential in chronic ulcers, to establish the difference between squamous cell carcinoma and pseudoepitheliomatous hyperplasia