A clinicopathological study of the oral lesions of Cowden disease.

BACKGROUND: Cowden disease is a multisystemic cancer predisposition disorder, inherited in an autosomal dominant pattern. Cutaneous and visceral lesions are either malignant tumours or are believed to represent hamartomatous growths. Mucocutaneous lesions are present on almost 100% of affected individuals. Nonetheless, some authors consider that the mucocutaneous manifestations of Cowden disease are, in reality, viral warts at distinct steps of evolution. There are only a few studies regarding the oral manifestations of Cowden disease. OBJECTIVES: To study the oral manifestations of Cowden disease in nine patients clinically and histopathologically. METHODS: The oral mucosal aspects of nine patients with clinical diagnosis of Cowden disease were photographed, and biopsies were taken from typical lesions. RESULTS: All biopsied lesions depicted histopathological aspects suggestive of viral warts at distinct steps of development: incipient, well established and in regression. CONCLUSION: Our findings seem to reinforce the poorly understood hypothesis that the mucocutaneous manifestations of Cowden disease are in reality viral warts. Additional studies are needed to explain why a syndrome caused by the mutation of a tumour suppression gene may cause propensity to warts.

Efficacy and safety of methotrexate in alopecia areata.

BACKGROUND: Alopecia areata is a chronic disorder of the hair follicles and nails, of unknown etiology, with clear autoimmune components and genetic factors. Several therapeutic options have been suggested; however, no treatment is able to modify the disease course. Methotrexate is an immunosuppressant used in various dermatoses and recently introduced as a therapeutic option for alopecia areata. OBJECTIVES: To evaluate the efficacy and safety of methotrexate in alopecia areata. METHODS: In a retrospective, non-controlled study, we evaluated 31 patients with alopecia areata in current or prior treatment with methotrexate to assess the therapeutic response according to sex, age, pattern of alopecia areata, disease duration, cumulative dose of methotrexate, use of systemic corticosteroids or other treatments, and drug safety. RESULTS: Regrowth greater than 50% was observed in 67.7% of patients, with the best responses observed in those with <5 years of disease progression (79%), age over 40 years (73.3%), male patients (72.8%), cumulative dose of methotrexate 1000-1500 mg, and multifocal alopecia areata (93%). Among patients receiving systemic corticosteroids in combination with methotrexate, 77.3% had greater than 50% regrowth, compared with 44.4% in those who used methotrexate alone. The therapeutic dose ranged from 10-25 mg/week. No patient had serious adverse effects. Relapse was observed in 33.3% of patients with more than 50% regrowth. CONCLUSION: Methotrexate appears to be a promising and safe medication for the treatment of severe alopecia areata when used alone or in combination with corticosteroids.

Efficacy and safety of methotrexate in alopecia areata

BACKGROUND: Alopecia areata is a chronic disorder of the hair follicles and nails, of unknown etiology, with clear autoimmune components and genetic factors. Several therapeutic options have been suggested; however, no treatment is able to modify the disease course. Methotrexate is an immunosuppressant used in various dermatoses and recently introduced as a therapeutic option for alopecia areata. OBJECTIVES: To evaluate the efficacy and safety of methotrexate in alopecia areata. METHODS: In a retrospective, non-controlled study, we evaluated 31 patients with alopecia areata in current or prior treatment with methotrexate to assess the therapeutic response according to sex, age, pattern of alopecia areata, disease duration, cumulative dose of methotrexate, use of systemic corticosteroids or other treatments, and drug safety. RESULTS: Regrowth greater than 50% was observed in 67.7% of patients, with the best responses observed in those with <5 years of disease progression (79%), age over 40 years (73.3%), male patients (72.8%), cumulative dose of methotrexate 1000-1500 mg, and multifocal alopecia areata (93%). Among patients receiving systemic corticosteroids in combination with methotrexate, 77.3% had greater than 50% regrowth, compared with 44.4% in those who used methotrexate alone. The therapeutic dose ranged from 10-25 mg/week. No patient had serious adverse effects. Relapse was observed in 33.3% of patients with more than 50% regrowth. CONCLUSION: Methotrexate appears to be a promising and safe medication for the treatment of severe alopecia areata when used alone or in combination with corticosteroids. .

Oral mucosal manifestations in some genodermatoses: correlation with cutaneous lesions.

The clinical picture of several genetic skin diseases may include the presence of oral mucosal lesions. These manifestations, however, have not been granted much attention in most dermatological publications. In this article, we fully review the oral mucosal lesions of tuberous sclerosis, dyskeratosis congenita, lipoidoproteinosis, Cowden disease, Darier's disease and pachyonychya congenita and compare these with their respective cutaneous lesions. Some dental aspects are discussed as well. This unifying approach may allow a better understanding of these oral lesions, avoiding obscure nomenclature and classification.

Ichthyosis associated with widespread tinea corporis: report of three cases.

Ichthyoses are a common group of keratinization disorders. A non-inflammatory generalized persistent skin desquamation is observed. It is characterized by increased cell turnover, thickening of the stratum corneum and functional changes of sebaceous and sweat glands. All of these favor fungal proliferation. Dermatophytes may infect skin, hair and nails causing ringworm or tinea. They have the ability to obtain nutrients from keratinized material. One of its most prevalent genera is Trichophyton rubrum. Although tineas and ichthyoses are quite common, the association of the two entities is rarely reported in the literature. Three cases of ichthyosis associated with widespread infection by T. rubrum are presented. Resistance to several antifungal treatments was responsible for worsening of ichthyosis signs and symptoms.

Ichthyosis associated with widespread tinea corporis: report of three cases / Ictiose associada a dermatofitose disseminada: relato de tres casos

Ichthyoses are a common group of keratinization disorders. A non-inflammatory generalized persistent skin desquamation is observed. It is characterized by increased cell turnover, thickening of the stratum corneum and functional changes of sebaceous and sweat glands. All of these favor fungal proliferation. Dermatophytes may infect skin, hair and nails causing ringworm or tinea. They have the ability to obtain nutrients from keratinized material. One of its most prevalent genera is Trichophyton rubrum. Although tineas and ichthyoses are quite common, the association of the two entities is rarely reported in the literature. Three cases of ichthyosis associated with widespread infection by T. rubrum are presented. Resistance to several antifungal treatments was responsible for worsening of ichthyosis signs and symptoms.

Ictioses são distúrbios da queratinização que se expressam com descamação cutânea generalizada persistente não inflamatória. Os pacientes que apresentam essa doença possuem aumentada renovação celular, maior espessura da camada córnea e alterações funcionais de glândulas sebáceas e sudoríparas, aspectos que parecem favorecer a infecção por dermatófitos. Dermatofitoses são infecções causadas por fungos ceratinofílicos, que acometem pele, pelos e unhas. Um dos gêneros mais prevalentes é o Trichophyton rubrum. Apesar das infecções fúngicas por dermatófitos e os quadros clínicos de ictiose serem muito comuns, são raros em literatura os relatos de associações dessas duas entidades. Apresentamos os relatos de três casos de ictiose associada a dermatofitose por T. rubrum. A ausência de resposta aos antifúngicos leva à piora clínica da ictiose.

Mucormycosis and chromoblastomycosis occurring in a patient with leprosy type 2 reaction under prolonged corticosteroid and thalidomide therapy.

Mucormycosis is an uncommon fungal infection caused by Mucorales. It frequently occurs in patients with neutropenia, diabetes, malignancy and on corticoid therapy. However, it is rare in patients with AIDS. Clinical disease can be manifested in several forms. The case reported illustrates the rare occurrence of chromoblastomycosis and mucormycosis in an immunosuppressed patient with multibacillary leprosy, under prolonged corticosteroid and thalidomide therapy to control leprosy type 2 reaction. Neutrophil dysfunction, thalidomide therapy and work activities are some of the risk factors in this case. Chromoblastomycosis was treated by surgical excision and mucormycosis with amphotericin B. Although the prognosis of mucormycosis is generally poor, in the reported case the patient recovered successfully. This case should alert dermatologists to possible opportunistic infections in immunosuppressed patients.

Mucormycosis and chromoblastomycosis occurring in a patient with leprosy type 2 reaction under prolonged corticosteroid and thalidomide therapy / Mucormicose e cromoblastomicose em um paciente com reação hansênica tipo II sob terapia prolongada com corticosteróide e talidomida

Mucormycosis is an uncommon fungal infection caused by Mucorales. It frequently occurs in patients with neutropenia, diabetes, malignancy and on corticoid therapy. However, it is rare in patients with AIDS. Clinical disease can be manifested in several forms. The case reported illustrates the rare occurrence of chromoblastomycosis and mucormycosis in an immunosuppressed patient with multibacillary leprosy, under prolonged corticosteroid and thalidomide therapy to control leprosy type 2 reaction. Neutrophil dysfunction, thalidomide therapy and work activities are some of the risk factors in this case. Chromoblastomycosis was treated by surgical excision and mucormycosis with amphotericin B. Although the prognosis of mucormycosis is generally poor, in the reported case the patient recovered successfully. This case should alert dermatologists to possible opportunistic infections in immunosuppressed patients.

Mucormicose é uma infecção fúngica incomum causada por Mucorales. Ocorre frequentemente em pacientes com neutropenia, diabetes, corticoterapia e condições malignas. Porém, é rara em pacientes com AIDS. A doença pode apresentar-se em diferentes formas. Este caso ilustra a rara ocorrência de mucormicose e cromoblastomicose em um paciente com hanseníase multibacilar, que estava sendo tratado com prednisona e talidomida devido a eritema nodoso (reação hansênica tipo II). Disfunção de neutrófilos, uso de talidomida e atividades profissionais são alguns fatores de risco neste caso. A cromoblastomicose foi tratada por excisão cirúrgica e a mucormicose com anfotericina B. Embora o prognóstico da mucormicose seja ruim, neste caso o tratamento foi bem sucedido. Este caso alerta dermatologistas para a possibilidade de infecções oportunistas em pacientes imunossuprimidos.

Case for diagnosis. Atypical fibroxanthoma.

Atypical fibroxanthoma is a rare cutaneous tumor found mainly in elderly people on sun-exposed areas of the body. Histologically, atypical fibroxanthoma is considered a malignant fibrous histiocytoma with bizarre neoplastic cells, marked pleomorphism, hyperchromatic nuclei and abundant mitoses. It must be differentiated from other skin tumors, usually by immunohistochemistry, since its diagnosis is made by exclusion.

Case for diagnosis / Caso para diagnóstico

Atypical fibroxanthoma is a rare cutaneous tumor found mainly in elderly people on sun-exposed areas of the body. Histologically, atypical fibroxanthoma is considered a malignant fibrous histiocytoma with bizarre neoplastic cells, marked pleomorphism, hyperchromatic nuclei and abundant mitoses. It must be differentiated from other skin tumors, usually by immunohistochemistry, since its diagnosis is made by exclusion.

O fibroxantoma atípico é um tipo de neoplasia cutânea maligna rara, encontrado principalmente em idosos, em áreas fotoexpostas. Na histologia, o fibroxantoma atípico é uma neoplasia fibro-histiocítica dérmica, de células fusiformes e epitelioides, algumas vezes bizarras, com acentuado pleomorfismo, apresentando núcleos hipercromáticos e mitoses abundantes. Deve ser diferenciado de outros tumores de pele, principalmente através da imunoistoquímica, já que seu diagnóstico é de exclusão.

Avaliação dos métodos de classificação do melasma de acordo com a resposta ao tratamento / Evaluation of melasma classification methods based on response to treatment

Introdução: O melasma pode ser classificado, através da lâmpada de Wood e da dermatoscopia, em epidérmico, dérmico e misto. Outros métodos de avaliação são o MASI e o MELASQol. Objetivos: O objetivo do estudo foi comparar os métodos não invasivos de classificação do melasma de acordo com a resposta ao tratamento. Métodos: Selecionadas 10 mulheres com melasma, foram submetidas a tratamento com hidroquinona 4% + tretinoína 0,05% + acetato de fluociolona 0,01% durante 90 dias, e avaliadas antes e após o tratamento por: dermatoscopia, lâmpada de Wood, MASI e MELASQol. Resultados: Não foram encontradas associações significativas entre MASI e classificação dermatoscópica, exame com lâmpada de Wood e MELASQol. O percentual médio de melhora após tratamento por MASI foi de 60,6% e por MELASQol, de 41,1%. À dermatoscopia observaram-se telangectasias na maioria das pacientes (60%), incluídas aquelas sem tratamento prévio. Conclusões: O MASI e o MELASQol são os instrumentos de avaliação que mais refletem a resposta ao tratamento. A classificação pela lâmpada de Wood não demonstrou correlação com a melhora do MASI. A utilização da dermatoscopia para classificação do melasma necessita de mais estudos, visto que os achados encontrados não se correlacionaram com a resposta esperada ao tratamento.

Introduction: Melasma can be classified as epidermal, dermal, or mixed using clinical measures such as Wood's lamp and dermoscopy. The Melasma Area Severity Index and the Melasma Quality of Life Scale are additional subjective classification methods. Objectives:To compare noninvasive melasma classification methods according to response to treatment. Methods: Ten women with melasma were treated with 4% hydroquinone + 0.05% tretinoin + 0.01% fluociolone acetate for 90 days. They were evaluated before and after treatment using dermoscopy, Wood's lamp, and both subjective measures. Results: No significant correlations were found between the Melasma Area Severity Index and dermoscopic classification, Wood's lamp examination and the Melasma Quality of Life Scale. The average percentage improvement after treatment was 60.6% in the severity index and 41.1% in the quality of life scale. Dermoscopy revealed telangiectasia in most patients (60%), including those without prior treatment. Conclusions: The subjective assessment tools best reflected the patients' response to treatment. The classification using Wood's lamp did correlate with improvement measured by the severity index. The use of dermoscopy for classifying melasma requires further research, since the findings were not correlated with the expected response to treatment.

Conector alternativo para diluição de anestésico e hidroxiapatita de cálcio para preenchimento cutâneo / Alternative connector for anesthetic and calcium hydroxyapatite dilution in the cutaneous filling technique

Várias opções de anestésicos são utilizadas para preenchimentos cutâneos. Recentemente foi descrita a combinação da hidroxiapatita de cálcio com lidocaína na mesma seringa utilizando o conector fêmea-fêmea Luer-lok. Descrevem-se outra opção de conector para realização dessa mistura, o instrumento e detalhes da associação.

A number of anesthetic options are available for use in cutaneous fillings.The combination of calcium hydroxyapatite with lidocaine in the same syringe using the female Luer Lok connector has been recently described.An additional connector option to combine these substances, the device itself, and the details of the association are described.