RESUMO
Erythema multiforme (EM) is a targetoid eruption with interface pathology often triggered by a hypersensitivity response to a variety of infections, most commonly herpes simplex virus. Hepatitis C virus is rarely associated with EM. We present a 37-year-old man with an 8-year history of severe EM unresponsive to valacyclovir, acitretin, and cyclosporin, and marginally responsive to high-dose prednisone. The eruption had cleared 6 years previously during treatment with interferon for his concurrent hepatitis C virus. Although his viral titer was undetectable, we initiated therapy with interferon and ribavirin. The patient responded dramatically within 2 months and remained clear of EM after 1 year of continued interferon therapy. This is the third case reported in the world literature documenting a response of EM to interferon, and the first case in which hepatitis C virus was undetectable in serum prior to interferon therapy.
Assuntos
Antineoplásicos/administração & dosagem , Eritema Multiforme/tratamento farmacológico , Interferon-alfa/administração & dosagem , Adulto , Eritema Multiforme/patologia , Humanos , Masculino , Indução de Remissão , Índice de Gravidade de DoençaAssuntos
Isoenzimas/análise , Paniculite/enzimologia , Deficiência de alfa 1-Antitripsina/diagnóstico , alfa 1-Antitripsina/análise , Eletroforese em Gel de Amido , Humanos , Isoenzimas/genética , Neutrófilos/patologia , Paniculite/etiologia , Paniculite/patologia , Fenótipo , alfa 1-Antitripsina/genética , Deficiência de alfa 1-Antitripsina/complicações , Deficiência de alfa 1-Antitripsina/enzimologiaRESUMO
Over 90 mutant alleles of the alpha-1-antitrypsin (AAT) gene are recognized and classified by mobility on an acid starch gel. The four major categories include: F=fast, M=medium, S=slow, Z=very slow. Among 41 reported cases of AAT panniculitis, most have the ZZ phenotype with AAT levels below normal. We report two cases of AAT panniculitis with MS phenotype and normal AAT levels. In addition, we review the pathophysiology, epidemiology, and extracutaneous manifestations of AAT disease and propose a diagnostic algorithm for ulcerative panniculitis. A 42-year-old man presented with a solitary plaque on the left thigh exacerbated by trauma or excessive activity. The lesion frequently suppurated with a yellowish oily material. Twenty years before, he had fractured his left femur which was repaired with a metal plate. X-rays, histology with special stains for organisms, and cultures were negative. AAT phenotype was MS and AAT value was normal. A 43-year-old woman presented with multiple plaques on the proximal extremities which suppurated with exercise or trauma. AAT phenotype was MS and AAT level was normal. Histologic exam for both patients showed a dense neutrophilic infiltrate with septal and lobular panniculitis and areas of necrobiosis in the lower reticular dermis.
Assuntos
Paniculite/diagnóstico , Paniculite/genética , Deficiência de alfa 1-Antitripsina/genética , alfa 1-Antitripsina/genética , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paniculite/sangue , Paniculite/classificação , Fenótipo , alfa 1-Antitripsina/análise , Deficiência de alfa 1-Antitripsina/sangue , Deficiência de alfa 1-Antitripsina/diagnósticoAssuntos
Antineoplásicos/efeitos adversos , Toxidermias/diagnóstico , Dermatoses da Mão/diagnóstico , Hidroxiureia/efeitos adversos , Doenças do Pênis/diagnóstico , Úlcera Cutânea/diagnóstico , Idoso , Antineoplásicos/administração & dosagem , Doença Crônica , Diagnóstico Diferencial , Toxidermias/etiologia , Toxidermias/patologia , Dermatoses da Mão/induzido quimicamente , Dermatoses da Mão/patologia , Humanos , Hidroxiureia/administração & dosagem , Leucemia Mieloide/tratamento farmacológico , Masculino , Doenças do Pênis/induzido quimicamente , Doenças do Pênis/patologia , Úlcera Cutânea/induzido quimicamente , Úlcera Cutânea/patologiaRESUMO
Basal cell carcinoma (BCC) is the most common cutaneous skin malignancy. BCC generally has a clinical course characterized by slow growth, minimal soft tissue invasiveness, and a high cure rate. Occasionally, however, BCC behaves aggressively with deep invasion, recurrence, and potential regional and distant metastasis. Several factors, including tumor size, duration, histology, and perineural spread, have been postulated as markers of the aggressive BCC phenotype. It is undetermined whether intrinsic biological factors within certain subsets of BCC predispose these tumors toward an inherently aggressive behavior, or whether any BCC with inadequate early management may assume this phenotype. Review of the pertinent literature on this topic suggests that both intrinsic biological factors and extrinsic management factors play a role in the development and progression of aggressive BCC.
Assuntos
Carcinoma Basocelular/patologia , Carcinoma Basocelular/terapia , Carcinoma Basocelular/irrigação sanguínea , Carcinoma Basocelular/metabolismo , Matriz Extracelular/metabolismo , Humanos , Metaloproteinases da Matriz/metabolismo , Recidiva , Fatores de RiscoRESUMO
Kaposi's sarcoma (KS) was described by Moritz Kaposi in 1872 and was known for an entire century as a rare disorder of older men usually of Eastern European, Mediterranean, and/or Jewish origin. In the early 1980s, the prevalence of KS began to increase dramatically and soon became the most common malignancy in patients with AIDS, especially those who were male homosexuals. In 1994, a new human herpesvirus (HHV) was found to be present in almost 100% of KS lesions. This virus was found to be a gammaherpesvirus, closely related to Epstein-Barr virus, and was designated HHV-8. Subsequently, HHV-8 DNA was found in almost all specimens of classic KS, endemic KS, and iatrogenic KS, as well as epidemic KS (ie, AIDS KS). It is now believed that HHV-8 is necessary, but not sufficient, to cause KS and that other factors such as immunosuppression play a major role. The use of highly active antiretroviral therapy (HAART) since 1996 has markedly reduced the prevalence of AIDS KS in western countries, but because 99% of the 40 million patients with AIDS in the world cannot afford HAART, KS is still a very common problem. Primary effusion lymphoma and multicentric Castleman's disease are also thought to be due to HHV-8. Although HHV-8 DNA has been described in a number of other cutaneous disorders, there is little evidence that HHV-8 is of etiologic significance in these diseases. The mechanism by which HHV-8 causes KS, primary effusion lymphoma, and multicentric Castleman's disease is not well understood but is thought to involve a number of molecular events, the study of which should further our understanding of viral oncology. (J Am Acad Dermatol 2002;47:641-55.) Learning objective: At the completion of this learning activity, participants should be familiar with Kaposi's sarcoma and other manifestations of human herpesvirus 8.
Assuntos
Humanos , Estudos Soroepidemiológicos , Fases de Leitura Aberta/fisiologia , /fisiologia , Hiperplasia do Linfonodo Gigante/virologia , Infecções por Herpesviridae/virologia , Produtos do Gene tat/genética , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/epidemiologia , Sarcoma de Kaposi/tratamento farmacológico , Sarcoma de Kaposi/virologia , Terapia Antirretroviral de Alta AtividadeRESUMO
UNLABELLED: Kaposi's sarcoma (KS) was described by Moritz Kaposi in 1872 and was known for an entire century as a rare disorder of older men usually of Eastern European, Mediterranean, and/or Jewish origin. In the early 1980s, the prevalence of KS began to increase dramatically and soon became the most common malignancy in patients with AIDS, especially those who were male homosexuals. In 1994, a new human herpesvirus (HHV) was found to be present in almost 100% of KS lesions. This virus was found to be a gammaherpesvirus, closely related to Epstein-Barr virus, and was designated HHV-8. Subsequently, HHV-8 DNA was found in almost all specimens of classic KS, endemic KS, and iatrogenic KS, as well as epidemic KS (ie, AIDS KS). It is now believed that HHV-8 is necessary, but not sufficient, to cause KS and that other factors such as immunosuppression play a major role. The use of highly active antiretroviral therapy (HAART) since 1996 has markedly reduced the prevalence of AIDS KS in western countries, but because 99% of the 40 million patients with AIDS in the world cannot afford HAART, KS is still a very common problem. Primary effusion lymphoma and multicentric Castleman's disease are also thought to be due to HHV-8. Although HHV-8 DNA has been described in a number of other cutaneous disorders, there is little evidence that HHV-8 is of etiologic significance in these diseases. The mechanism by which HHV-8 causes KS, primary effusion lymphoma, and multicentric Castleman's disease is not well understood but is thought to involve a number of molecular events, the study of which should further our understanding of viral oncology. (J Am Acad Dermatol 2002;47:641-55.) LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with Kaposi's sarcoma and other manifestations of human herpesvirus 8.