Consensus on the treatment of hidradenitis suppurativa - Brazilian Society of Dermatology.

Hidradenitis suppurativa is a chronic immune mediated disease of universal distribution that causes great damage to the quality of life of the affected individual, whose prevalence is estimated at 0.41% in the Brazilian population. The objective of this work was update on physiopathogenesis, diagnosis and classification of hidradenitis suppurativa and to establish therapeutic recommendations in the Brazilian reality. It was organized as a work group composed of eight dermatologists from several institutions of the country with experience in the treatment of hidradenitis suppurativa and carried out review on the topic. Recommendations were elaborated and voted by modified Delphi system and statistical analysis of the results was performed. The Brazilian consensus on the clinical approach of hidradenitis suppurativa had the support of the Brazilian Society of Dermatology.

Consensus on the treatment of hidradenitis suppurativa - Brazilian Society of Dermatology

Abstract: Hidradenitis suppurativa is a chronic immune mediated disease of universal distribution that causes great damage to the quality of life of the affected individual, whose prevalence is estimated at 0.41% in the Brazilian population. The objective of this work was update on physiopathogenesis, diagnosis and classification of hidradenitis suppurativa and to establish therapeutic recommendations in the Brazilian reality. It was organized as a work group composed of eight dermatologists from several institutions of the country with experience in the treatment of hidradenitis suppurativa and carried out review on the topic. Recommendations were elaborated and voted by modified Delphi system and statistical analysis of the results was performed. The Brazilian consensus on the clinical approach of hidradenitis suppurativa had the support of the Brazilian Society of Dermatology.

Morbihan disease: a therapeutic challenge

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Morbihan disease: a therapeutic challenge.

Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Analysis of mutations in the PIK3CA and FGFR3 genes in verrucous epidermal nevus.

Verrucous epidermal nevi are congenital hamartomas composed of keratinocytes and may occur alone or in association with developmental abnormalities. A close relationship between variations in the PIK3CA and FGFR3 genes and the appearance of nevi has been recently reported. Based on that, we performed molecular assays for the identification of E542K, E545G/K and H1047R mutations in the PIK3CA gene and of the R248C mutation in the FGFR3 gene. Interestingly, during the amplification process, we did not observe the PCR product of exon 9 of the PIK3CA gene, a region comprising amino acids 542-545. This strongly suggests the occurrence of a microdeletion of that region and indicates a possible allelic variant, which has not yet being described in the literature.

Analysis of mutations in the PIK3CA and FGFR3 genes in verrucous epidermal nevus / Analise de mutacoes nos genes PIK3CA e FGFR3 em caso de nevo epidermico verrucoso

Verrucous epidermal nevi are congenital hamartomas composed of keratinocytes and may occur alone or in association with developmental abnormalities. A close relationship between variations in the PIK3CA and FGFR3 genes and the appearance of nevi has been recently reported. Based on that, we performed molecular assays for the identification of E542K, E545G/K and H1047R mutations in the PIK3CA gene and of the R248C mutation in the FGFR3 gene. Interestingly, during the amplification process, we did not observe the PCR product of exon 9 of the PIK3CA gene, a region comprising amino acids 542-545. This strongly suggests the occurrence of a microdeletion of that region and indicates a possible allelic variant, which has not yet being described in the literature.

Os nevos epidérmicos verrucosos são hamartomas congênitos compostos por queratinócitos, que podem ocorrer isolados ou associados a alterações do desenvolvimento. Com a recente observação de uma relação estreita entre variações nos genes PIK3CA e FGFR3 e o aparecimento do nevo, realizamos ensaios moleculares para identificação das mutações E542K, E545G/K e H1047R do gene PIK3CA, e R248C do gene FGFR3. Interessantemente, durante o processo de amplificação não observamos o produto da PCR do exon 9 do gene PIK3CA, região que compreende aos aminoácidos 542-545, sugerindo fortemente a ocorrência de uma microdeleção da região e indicando uma provável variante alélica ainda não descrita na literatura.

Complicação de preenchimento cutâneo após tratamento de hepatite C com interferon e ribavirina / Dermal filling complication after hepatitis C treatment with interferon and ribavirin

Relata-se o caso de paciente portadora de hepatite C, com surgimento de granuloma por corpo estranho nos sulcos nasolabiais e glabela, cinco anos após submeter-se a aplicação de preenchedor cutâneo permanente. A paciente fez uso de interferon e ribavirina para tratar hepatite C durante seis meses, com o surgimento dos granulomas 30 dias após o fim do tratamento. Realizada então terapia com corticoide oral, havendo melhora gradativa do quadro cutâneo.

This article describes the case of a female hepatitis C patient who presented with a foreign body granuloma in the nasolabial folds and glabella five years after receiving an application of permanent dermal filler. The patient used interferon and ribavirin for treating hepatitis C for six months, and the granulomas appeared 30 days after the end of the treatment. Treatment with oral steroids was carried out, with a gradual improvement in the appearance of the skin.

Association of oral acyclovir and imiquimod for the treatment of hypertrophic genital herpes simplex in HIV positive patients: report of two cases.

Chronic herpes simplex can present as exuberant clinical lesions, especially in HIV patients. The most probable mechanism of formation of these lesions is the invasion of the epidermal CD4 T cells by the herpes simplex virus. Due to the therapeutic difficulties and the high rates of treatment failure amongst these patients, new drugs are currently being discussed on the literature. Studies based on the immunophatology of these lesions have suggested that imiquimod might work as an adjuvant therapy to the antiviral drugs. Here we present two cases of excellent response to treatment with topical imiquimod as an adjuvant drug to acyclovir.

Use of cyclosporin in a patient with hepatitis C and pustular psoriasis.

Cyclosporine has been contraindicated in patients with chronic infections such as infection with hepatitis C because of its immunosuppressive effect. Recent studies have shown however that cyclosporine suppresses viral replication and thus cannot exacerbate infection with hepatitis C when employed for treating patients with psoriasis. We present the case of a female patient with psoriasis for 30 years and hepatitis C for 20 years, with diffuse circinate lesions. Improvement in the skin condition and liver enzymes was obtained with the use of cyclosporine, with no adverse effect.

Associação de aciclovir oral e imiquimode no tratamento de herpes simples genital hipertrófico em paciente com sida: relato de dois casos / Association of oral acyclovir and imiquimod for the treatment of hypertrophic genital herpes simplex in HIV positive patients: report of two cases

Herpes simples crônico é doença capaz de gerar apresentações exuberantes, principalmente em pacientes com sida. O provável mecanismo da formação dessas lesões hiperplásicas consiste na invasão viral dos linfócitos T CD4 situados na epiderme. Diante das dificuldades terapêuticas e da grande taxa de insucesso do tratamento nesses pacientes, novas terapias têm sido citadas na literatura atualmente. O conhecimento da imunopatologia ratificou o mecanismo pelo qual o imiquimode poderia ser eficaz como terapia adjuvante aos antivirais. Relatamos aqui dois casos de pacientes tratados com aciclovir associado ao imiquimode tópico que obtiveram resposta clínica excepcional.

Chronic herpes simplex can present as exuberant clinical lesions, especially in HIV patients. The most probable mechanism of formation of these lesions is the invasion of the epidermal CD4 T cells by the herpes simplex virus. Due to the therapeutic difficulties and the high rates of treatment failure amongst these patients, new drugs are currently being discussed on the literature. Studies based on the immunophatology of these lesions have suggested that imiquimod might work as an adjuvant therapy to the antiviral drugs. Here we present two cases of excellent response to treatment with topical imiquimod as an adjuvant drug to acyclovir.

Uso da ciclosporina em paciente portador de hepatite C e psoríase pustulosa / Use of cyclosporin in a patient with hepatitis C and pustular psoriasis

A ciclosporina tem sido contraindicada nos pacientes com infecções crônicas, como a infecção pelo vírus da hepatite C, devido ao seu efeito imunossupressor. No entanto, estudos recentes têm demonstrado que a ciclosporina suprime a replicação viral e pode, desta forma, não exacerbar a infecção pelo vírus da hepatite C, quando administrada como tratamento para pacientes com psoríase. Apresentamos o caso de uma paciente portadora de psoríase há 30 anos e hepatite C há 20 anos, com lesões circinadas difusas, que apresentou melhora cutânea e das enzimas hepáticas com o uso da ciclosporina, sem apresentar nenhum efeito adverso.

Cyclosporine has been contraindicated in patients with chronic infections such as infection with hepatitis C because of its immunosuppressive effect. Recent studies have shown however that cyclosporine suppresses viral replication and thus cannot exacerbate infection with hepatitis C when employed for treating patients with psoriasis. We present the case of a female patient with psoriasis for 30 years and hepatitis C for 20 years, with diffuse circinate lesions. Improvement in the skin condition and liver enzymes was obtained with the use of cyclosporine, with no adverse effect.