Abstract Bullous melanoma represents a rare variant of melanoma, especially in patients without underlying bullous cutaneous disease. Few cases have been described in the literature, including cases of melanoma in patients with bullous epidermolysis or Hailey-Hailey disease. The histopathological diagnosis of bullous melanoma does not show any difficulties, except for the measurement of the Breslow index. The rarity of this case, the dilemma of how to measure the Breslow index and the importance of an early diagnosis motivated this report.

Abstract: Cutaneous metastases are uncommon in daily practice, although very important, since they may be the first manifestation of an undiscovered primary neoplasm or the first indication of recurrence. Cutaneous metastases from the breast are the most frequent in women and cutaneous metastases from the lung are the most frequent in men. Thyroid carcinoma, despite representing the most frequent endocrine neoplasm, is considered a rare neoplasm, corresponding to 1% of malignant neoplasms diagnosed. Cutaneous metastases from follicular carcinoma are rare and occur mainly in the head and neck area. We report a case of cutaneous metastasis in a patient with follicular thyroid carcinoma and breast carcinoma. Because of the association of these two neoplasms, the possibility of Cowden Syndrome - multiple hamartoma syndrome - was raised, but was excluded by genetic analysis of PTEN gene.

Abstract: Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of Sweet's syndrome. Although the etiology is unknown, there are reports of association with infections, neoplasias, autoimmune diseases and medications. Histopathology shows a dense neutrophilic inflammatory infiltrate in the dermis. Treatment is based on the administration of systemic corticosteroids; however, a combination of medications is useful, given the frequency of relapses. The authors report a classic and clinically exuberant case of neutrophilic dermatosis of the dorsal hands, with excellent response to oral dapsone treatment, and offer a brief literature review.

AbstractErythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of unknown etiology. It is believed to be due to deposition of immune complexes in the vessels. Clinically it is manifested as erythematous violaceous papules and nodules, isolated or confluent with hardened consistency, symmetrical, usually located on the extensor surface of the extremities, particularly over the joints. Diagnosis is based on clinical and histological findings. We report the case of a woman, 71 years old, with erythematous violaceous nodules on the hands, elbows, back and legs, beginning two years ago, with pain and itching. Histopathological analysis revealed leukocytoclastic vasculitis, confirming the clinical suspicion. Laboratory tests revealed hypothyroidism. We report the case because of its rarity, with subsequent review of the literature.

Abstract TNF alpha antagonist-induced lupus-like syndrome is a rare condition which predominantly affects women (4:1). The average age of onset is 46-51 years. It occurs after exposure to TNF alpha antagonist and disappears after discontinuation of such agents. The pathogenic mechanism for development of the TNF alpha antagonist-induced lupus-like syndrome is not fully defined. It is believed that the medication induces apoptosis, leading to an accumulation of nucleosomal antigens of apoptotic cells. This would cause autoantibodies to be produced by susceptible individuals. The most common cutaneous manifestations include maculopapular exanthem, malar rash, alopecia, photosensitivity and, more rarely, vasculitis. Extracutaneous manifestations include: fever, weight loss, arthritis or arthralgia, myositis and hematological abnormalities. Antinuclear antibody may be positive in 80% of cases and anti-histone antibody is considered a disease marker for TNF alpha antagonist-induced lupus-like syndrome. Treatment corresponds to drug discontinuation. We report a rare case of sub-acute cutaneous lupus erythematosus with leukocytoclastic vasculitis induced by adalimumab in a 42-year-old patient.

A poroqueratose é uma desordem da queratinização epidérmica caracterizada por placas anulares com centro atrófico e bordas hiperqueratóticas, e inclui um grupo heterogêneo de desordens que são na sua maioria herdadas de forma autossômica dominante. O presente caso refere-se a uma paciente feminina de 5 anos de idade, com poroqueratose de Mibelli confirmada histologicamente. A raridade desta desordem, a exuberância clínica e o caráter destrutivo das lesões, o acometimento facial e mucoso pouco usuais nesta forma de poroqueratose e o início na infância precoce motivaram este relato.

Porokeratosis is a disorder of epidermal keratinization characterized by annular plaques with an atrophic center and hyperkeratotic edges, and includes a heterogeneous group of disorders that are mostly inherited in an autosomal dominant form. This report describes a 5 year-old female patient, with porokeratosis of Mibelli confirmed histopathologically. The rarity of this disorder, its clinical exuberance and the destructive character of the lesions, as well as the facial and mucosal involvements, unusual in this form of porokeratosis, and also its onset in early childhood motivated this report.

Relata-se o caso de uma paciente com diagnóstico de angioqueratoma pseudolinfomatoso acral, conhecido por sua sigla em inglês APACHE - Acral Pseudolymphomatous Angiokeratoma of Children. É uma doença cutânea benigna, rara, de etiologia desconhecida, caracterizada por múltiplas pápulas e nódulos eritêmato-violáceos assintomáticos, de localização geralmente unilateral e acral. Atualmente, questiona-se esta denominação, já que há relatos na literatura do quadro em adultos e em outras localizações. Clinicamente, é similar a um angioqueratoma, porém, histologicamente, corresponde a um tipo distinto de pseudolinfoma. O estudo imuno-histoquímico é necessário para diferenciação dos linfomas cutâneos.

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

FUNDAMENTOS: O carcinoma basocelular é a forma mais comum de câncer em humanos. OBJETIVOS: Identificar a epidemiologia do carcinoma basocelular em Taubaté-SP e verificar possível associação entre a topografia e os diferentes subtipos histológicos deste tumor. MÉTODOS: Estudo transversal e descritivo realizado no Hospital Universitário de Taubaté entre 01/01/08 e 31/12/09. Foram incluídos neste estudo indivíduos com diagnóstico confirmado de carcinoma basocelular, de ambos os gêneros, sem restrição quanto a idade. As variáveis estudadas foram ocorrência do carcinoma basocelular, topografia, subtipo histológico, cor da pele, idade e gênero. Foi utilizado o teste do qui-quadrado para identificar associação entre o subtipo histológico e a topografia, e o teste t de student para comparar a média de idade de acometimento entre os diferentes subtipos histológicos. RESULTADOS: Foram incluídos 239 indivíduos.A média de idade da amostra foi de 68,0 anos. O gênero masculino foi o mais prevalente(57,7%), assim como a pele branca(87,1%).O subtipo histológico predominante foi o nodular(34,7%), seguido do subtipo superficial.A localização mais frequente foi a região de cabeça e pescoço(área fotoexposta), com predomínio da região nasal. Exceção para o subtipo superficial que mostrou forte associação com áreas cobertas como o tronco. A média de idade de incidência do carcinoma basocelular superficial também diferiu dos demais subtipos histológicos, 63.0 e 69.0 anos, respectivamente. CONCLUSÃO: Os resultados sugerem associação do subtipo histológico superficial com faixas etárias mais jovens e áreas cobertas, relacionando o mesmo com um padrão de exposição solar intermitente, mais semelhante ao padrão de fotocarcinogênese do melanoma.

BACKGROUND: Basal cell carcinoma is the most common form of cancer in humans. OBJECTIVES: To identify the epidemiology of basal cell carcinoma in Taubaté-SP and verify a possible association between topography and the different histological subtypes of this tumor. METHODS: This was a cross-sectional study conducted at The University Hospital of Taubaté between 01/01/08 and 12/31/09. The study included patients with a confirmed diagnosis of basal cell carcinoma, of both genders, without age restrictions. The variables studied were incidence of basal cell carcinoma, topography, histological subtype, skin color, age and gender. We employed the chi-square test to identify the association between histological subtype and topography, and the student's t test to compare the mean age of onset for the different histological subtypes. RESULTS: The study included 239 individuals. The mean age of the sample was 68.0 years. Male subjects (57.7%) and whites (87.1%) predominated in the study. The predominant histological subtype was nodular (34.7%), followed by the superficial subtype. The most frequent sites of involvement were the head and neck (areas exposed to light), with predominance of the nasal region. The superficial subtype was an exception, as it showed a strong association with unexposed areas like the trunk. The mean age of onset of superficial basal cell carcinoma also differed from that of the other histological subtypes, 63.0 and 69.0 years, respectively. CONCLUSION: The results of this study suggest an association of the superficial histological subtype with younger patients and unexposed areas of the body, linking this type of tumor with a pattern of intermittent sun exposure, more similar to the standard photocarcinogenesis of melanoma.