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.

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.

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.