Abstract: The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.

Chronic mucocutaneous candidiasis is a rare disorder characterized by persistent and recurrent infections by Candida due to changes in cellular immunity and may be associated with autoimmune endocrine disorders. It is refractory to the usual antifungal treatments, which merely control it with imidazole derivatives. This reports the case of a 50-year-old female patient who referred vaginal discharge associated with vulvar ulcerated lesions and whitish plaques on oral and genital mucous membranes of onset in adolescence besides cutaneous horns in nipples. The clinical picture, family history, culture and anatomopathological studies were consistent with chronic infection by candida. Treatment with systemic antifungals obtained partial response of lesions characterizing a clinical picture of Chronic Mucocutaneous Candidiasis.

O carcinoma basocelular é a neoplasia maligna cutânea mais comum em humanos, localizando-se, frequentemente, em áreas expostas e em indivíduos de pele clara. Relata-se o caso de uma paciente de 62 anos, faiodérmica, com múltiplas lesões de bordas discretamente elevadas, eritemato-acastanhadas na região pubiana, cujo diagnóstico clínico foi carcinoma basocelular pigmentado, confirmado através do estudo histopatológico. A imunoistoquímica das lesões foi negativa para a pesquisa de papiloma vírus

Basal cell carcinoma is the most common type of malignant cutaneous neoplasm in humans, being more frequently located in exposed areas and in fair-skinned individuals. It is reported the case of a 62-yearold female patient, brown-skinned ,with multiple lesions with edges slightly raised, reddish-brown in the pubic region, whose clinical diagnosis was pigmented basal cell carcinoma, confirmed by histopathology. Immunohistochemistry of the lesions was negative for the detection of papilloma virus

INTRODUÇÃO: Em 1997, após a realização de estudo multicêntrico, duplo cego e randomizado, em nove centros de tratamento de hanseníase na Índia, o Ministério da Saúde adotou o esquema alternativo dose única ROM para casos de lesão única, paucibacilar, sem nervo periférico afetado, índice baciloscópico negativo, em Centros de Referência da doença no Brasil. O estudo se propôs a avaliar a efetividade do esquema ROM em pacientes tratados no período de 1997 a 1999 no Serviço de Dermatologia da Santa Casa de Vitória. MÉTODOS: Foram selecionados e tratados com o esquema ROM, 54 pacientes das formas indeterminada e tuberculóide. Estes pacientes foram convocados de março a outubro de 2006 para reavaliação clínica. RESULTADOS: Vinte e nove pacientes avaliados (85,2%; IC95%: 70-100,4) estavam curados, cinco (14,7%; IC95%: 7,4-22,0) recidivaram e 20 pacientes não retornaram; porém, não havia outra notificação de reingresso ao tratamento no banco de dados da Secretaria Estadual de Saúde. CONCLUSÕES: O estudo evidenciou taxa de cura de 90,8% e taxa bruta de recidiva de 9,2%, após período de sete a nove anos da dose ROM. O tratamento alternativo ROM demonstrou melhor efetividade para lesão única menor que quatro centímetros e aparecimento há menos de cinco anos.

INTRODUCTION: In 1997, after obtaining a combined multi-state double-blind randomly controlled clinical trial study from nine Indian centers involved in the treatment of Hansen's Disease, the Ministry of Health adapted the single dose ROM Therapy approach in those cases involving the treatment of a single skin lesion, paucibacillary leprosy without evidence of peripheral nerve trunk involvement and indication of negative baciloscope, in the Referral Centers in Brazil. The study aimed to evaluate the effectiveness of the single dose ROM Therapy approach in those patients who were treated from the period of 1997 to 1999 in the Ambulatory Dermatologic Unit in the Hospital in Vitória, ES. METHODS: Fifty-four patients with tuberculoid and indeterminate leprosy were selected and treated with the single dose ROM Therapy approach. These patients were contacted from March 2006 up and until October 2006 for further clinical reevaluation. RESULTS: From the studies conducted, the following results were found to exist: 29 patients (85,2%; 95%CI: 70-100,4) were cured, 5 patients (14,7%; 95%CI: 7,4-22,0) relapsed, and 20 patients didn't return; however, there are no additional records of any notification of other treatment(s) in the State Department of Health's informational data banks. CONCLUSIONS: The study demonstrated a rate of cure of 90.8% and a rate of relapse of 9.2% after a period of seven to nine years using the single dose ROM Therapy approach. Additionally, this alternative treatment further demonstrated a better effectiveness for a single skin lesion smaller than four centimeters and with an appearance in less than five years.

Botriomicose é infecção bacteriana crônica, granulomatosa, supurativa, que afeta a pele e os tecidos subcutâneos. Caracteriza-se por lesão cutânea com múltiplas fístulas, que drena secreção purulenta, na qual se encontram grãos branco-amarelados, PAS-positivos. O principal agente etiológico é o Staphylococcus aureus, e outros germes menos frequentes são Pseudomonas aeruginosa, Escherichia coli, isolados ou associados. Os autores relatam um caso da doença, com lesões exuberantes de consistência fibrosa e múltiplos orifícios fistulosos dos quais drenavam grãos branco-amarelados, localizadas na perna direita e provocando aumento de volume do membro, que tiveram resolução com sulfametoxazol- trimetoprim.

Botryomycosis is a chronic, granulomatous, suppurative bacterial infection involving the skin and subcutaneous tissues. The skin lesion is characterized by multiple fistulae draining purulent secretion with white-yellowish grains PAS positive. The main etiologic agent is Staphylococcus aureus and other less frequent pathogens are Pseudomonas aeruginosa and Escherichia coli, isolated or associated. The authors report a case of this disease with exuberant lesions on the right leg and increased limb volume, fibrous consistency and multiple fistula orifices draining white-yellowish grains. Resolution was achieved with sulphamethoxazole-trimethoprim.

Paciente de 29 anos, do sexo feminino, com aparecimento há quatro anos de pápulas assintomáticas, eritêmato-acastanhadas, no antebraço direito. Duas das lesões foram biopsiadas para exames histopatológico e imuno-histoquímico. A paciente foi inicialmente tratada com corticoterapia intralesional, sem melhora. Procedeu-se, então, à exérese das lesões com resolução completa do quadro.

A 29-year-old female patient with asymptomatic red-brown papules that had arisen four years before in her right forearm. Two lesions were removed for histopathological and immunohistochemical exams. Intralesional corticosteroid injection resulted in no improvement and the lesions were subsequently surgically excised with complete resolution of the condition.

O líquen escleroso e atrófico é doença crônica da pele que se apresenta clinicamente de forma variada. Pode acometer mulheres e, menos freqüentemente, homens e crianças, sendo a área mais afetada a região anogenital. Há forte associação com doenças auto-imunes, e estudos imunogenéticos têm revelado associação com o HLA DQ7. Raramente acomete áreas extragenitais; entretanto, os autores relatam dois casos da doença, com localizações atípicas (extragenitais) e fazem uma abordagem terapêutica distinta. O uso tópico de corticoterapia de alta potência é relatado, porém, com riscos; enquanto agentes imunomoduladores tópicos são descritos com boa resposta clínica e com menor risco de atrofia.

Lichen sclerosus et atrophicus is a chronic skin disease with multiple clinical presentations. It affects women, and less frequently men and children. The disease is usually located in the anal and genital regions. There is a strong association with autoimmune diseases, and immunogenetic studies demonstrated an association with HLA DQ7. The disease rarely affects extra-genital regions. The authors report two cases of lichen sclerosus et atrophicus in atypical locations (extra-genital), in which different management was used. High potency topical corticosteroid therapy is reported, with risks, however. Topical immunomodulating agents are described, obtaining favorable clinical responses and decreased risk of atrophy.

Descreve-se caso clínico de paciente tratado de hanseníase dimorfa, que utilizou automedicação, com doses variadas de corticosteróide oral, por longo período, devido à neuropatia hansênica. Desenvolveu lesão nódulo-cística, amarelada, com algumas crostas, envolta por halo eritêmato-acastanhado, na face interna da perna esquerda. O exame micológico direto confirmou a hipótese diagnóstica de feo-hifomicose.

We report a patient suffering from dimorphic Hansen's disease who has self medicated his neuropathy with oral corticosteroid for a long time. A yellowish, nodular-cystic lesion partially topped with crusting, surrounded by a brownish red halo, was noted on the inner aspect of his left leg. Direct mycological examination confirmed the hypothesis of pheohyphomycosis.

As epidermólises bolhosas são dermatoses bolhosas congênitas que levam à formação de bolhas espontaneamente ou após trauma. São reconhecidos três grupos de da doença, de acordo com o segundo consenso internacional: simples, juncional e distrófica. Nas formas distróficas, o defeito genético deve-se à mutação no gene COL7A1, responsável pela codificação do colágeno VII, principal constituinte das fibrilas de ancoragem, que participam na aderência da lâmina densa à derme. Os autores relatam o caso de paciente do sexo feminino, de 15 anos, apresentando ulcerações nas pernas, bolhas serosas e lesões atrófico-acastanhadas nos braços e tronco. Foram observadas distrofias ungueais e alterações dentárias, iniciadas a partir do nascimento. O exame histopatológico da bolha revelou quadro compatével com epidermólise bolhosa, que, associado aos dados clínicos, permitiram a classificação do caso na forma distrófica recessiva mitis.

Epidermolysis bullosa are congenital bullous dermatoses that lead to spontaneous or post-traumatic formation of blisters. There are three recognized disease groups, according to the second international consensus: simplex, junctional and dystrophic. The genetic defect of the dystrophic forms is due to a mutation in the COL7A1 gene, which is responsible for codifying collagen VII, the main representative of anchoring fibrils, which participate in the adherence of the "lamina densa" to the dermis. The authors describe a case of a 15 year-old female patient who presented ulcers on her legs, serous blisters and atrophic scars on her arms and body. Dystrophic ungual and dental abnormalities had also been observed since her birth. Blister histopathological examination was compatible with epidermolysis bullosa, which, in association with clinical data, allowed the classification of recessive distrophic epidermolysis bullosa.

A piedra branca é infecção fúngica do pêlo, rara, causada pelo Trichosporon beigelii, caracterizada por nódulos amarelados nos pêlos genitais, bigode, barba, axilas e raramente, no couro cabeludo. O estudo registra 15 casos, diagnosticados em serviço de dermatologia de Vitória, durante cinco anos, todos de pacientes do sexo feminino, com nódulos nos pêlos do couro cabeludo. Os autores supõem que o clima quente e úmido da região e o hábito de as pacientes utilizarem cremes recondicionadores nos fios dos cabelos tenham favorecido a infecção. O umedecimento dos fios com água facilita a visualização dos nódulos.

White Piedra is a rare fungal infection of the hair shaft, caused by a yeast-like fungus (Trichosporon beigelii), characterized by yellow nodules on the pubic hair, moustache, beard and hair on the axillae and scalp. The objective was to register the presence of 15 cases of White Piedra in Espírito Santo diagnosed at the Dermatology Service in Vitória, over five years. All patients were female with nodules in the scalp hair, 13 were of mixed race and ten were between two and six years old. The hot and humid climate of the region together with the patients' habits regarding use of conditioning creams favored the infection. Moistening the hair with water can facilitate visualization of the nodules.