RESUMO
BACKGROUND: Atopic dermatitis (AD) is associated with a dysregulation of the skin barrier and may predispose to the development of secondary allergic conditions, such as asthma. Tmem79ma/ma mice harbor a mutation in the gene encoding Transmembrane Protein 79 (or Mattrin), which has previously been associated with AD. As a result of the Tmem79 gene mutation, these mice have a defective skin barrier and develop spontaneous skin inflammation. In this study, Tmem79ma/ma mice were assessed for the underlying immunological response in the development of spontaneous skin and lung inflammation. METHODS: Development of spontaneous skin and lung inflammation in Tmem79ma/ma mice was analyzed. We further investigated susceptibility to cutaneous Staphylococcus aureus infection. Tmem79ma/ma were crossed to IL-17A-deficient mice to address the contribution of IL-17A to spontaneous skin and lung disease. RESULTS: Tmem79ma/ma mice developed IL-17A-dependent spontaneous AD-like inflammation and were refractory to S aureus infection. Mutant mice progressed to airway inflammation subsequent to the occurrence of dermatitis. The progression from skin to lung disease is dependent on adaptive immunity and is facilitated by cutaneous expansion of Th17 and TCRγδ T cells. CONCLUSION: Mice lacking Tmem79/Mattrin expression have a defective skin barrier. In adulthood, these mice develop dermatitis with secondary progression to lung inflammation. The development of skin and lung inflammation is IL-17A-dependent and mediated by TCRγδ T cells.
Assuntos
Dermatite Atópica , Interleucina-17 , Pneumonia , Animais , Dermatite Atópica/genética , Modelos Animais de Doenças , Interleucina-17/genética , Proteínas de Membrana/genética , Camundongos , Pneumonia/genética , PeleRESUMO
BACKGROUND: Genital or vulval lichen planus (VLP) may have a disabling effect on a patient's quality of life. Evidence-based management guidelines are lacking for VLP. OBJECTIVE: We sought to review clinical presentation and treatment of patients who received a diagnosis of VLP. METHODS: The 100 consecutive patients who received a diagnosis of VLP at Mayo Clinic between January 1, 1997, and December 31, 2015, were reviewed retrospectively. Descriptive statistics were used for data analysis. Fisher's exact test and the Wilcoxon rank sum test were used for analysis of categorical and continuous variables, respectively. All statistical tests were 2 sided, with the α level set at .05 for statistical significance. RESULTS: The time to diagnosis for 49% of patients was more than 1 year. Three patients (3%) had vulval dysplasia, including invasive squamous cell carcinoma. Sixty-eight patients (68%) had multisite lichen planus disease. Eleven patients (11%) had disease remission. Dermatology was the lead specialty for 9 of these cases of remission. LIMITATIONS: This was a retrospective, small-cohort study. CONCLUSION: A low frequency of disease remission was seen in patients with VLP. Patients with lichen planus benefit considerably from dermatology consultation. Further research is warranted to establish high-quality, evidence-based guidelines for multidisciplinary management of this challenging disease.
Assuntos
Líquen Plano , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Líquen Plano/diagnóstico , Líquen Plano/terapia , Pessoa de Meia-Idade , Estudos Retrospectivos , Doenças da Vulva/diagnóstico , Doenças da Vulva/terapiaRESUMO
Pediatric genital mucocutaneous diseases are rare. A retrospective review was performed of children presenting with symptomatology of genital dermatoses to a hospital-based dermatology service. This study highlights that the range of genital diseases in children is not as broad as in adults. The diagnosis of genital mucocutaneous disease in children is usually genital lichen sclerosus, but other diagnoses should be considered.
