We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first line therapy option for moderate to severe pemphigus.

No abstract

PURPOSE More than 80% of patients who undergo sentinel lymph node (SLN) biopsy have no nodal metastasis. Here, we describe a model that combines clinicopathologic and molecular variables to identify patients with thin- and intermediate-thickness melanomas who may forgo the SLN biopsy procedure because of their low risk of nodal metastasis. PATIENTS AND METHODS Genes with functional roles in melanoma metastasis were discovered by analysis of next-generation sequencing data and case-control studies. We then used polymerase chain reaction to quantify gene expression in diagnostic biopsy tissue across a prospectively designed archival cohort of 754 consecutive thin- and intermediate-thickness primary cutaneous melanomas. Outcome of interest was SLN biopsy metastasis within 90 days of melanoma diagnosis. A penalized maximum likelihood estimation algorithm was used to train logistic regression models in a repeated cross-validation scheme to predict the presence of SLN metastasis from molecular, clinical, and histologic variables. RESULTS Expression of genes with roles in epithelial-to-mesenchymal transition (glia-derived nexin, growth differentiation factor 15, integrin-β3, interleukin 8, lysyl oxidase homolog 4, transforming growth factor-β receptor type 1, and tissue-type plasminogen activator) and melanosome function (melanoma antigen recognized by T cells 1) were associated with SLN metastasis. The predictive ability of a model that only considered clinicopathologic or gene expression variables was outperformed by a model that included molecular variables in combination with the clinicopathologic predictors Breslow thickness and patient age (area under the receiver operating characteristic curve, 0.82; 95% CI, 0.78 to 0.86; SLN biopsy reduction rate, 42%; negative predictive value, 96%). CONCLUSION A combined model that included clinicopathologic and gene expression variables improved the identification of patients with melanoma who may forgo the SLN biopsy procedure because of their low risk of nodal metastasis.

Background Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with an increased mortality rate. Objective To determine the incidence and mortality rate of patients with bullous pemphigoid. Methods Eighty-seven residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 – December 2009. Incidence and mortality rate were compared to age- and sex-matched control patients from the same geographic area. Results The adjusted incidence of BP was 2.4 per 100,000 person-years (95% CI, 1.9–2.9). Incidence of BP increased significantly with age (P<.001) and over time (P=0.034). Trend tests indicate increased diagnosis of localized disease (P=.006) may be a contributing factor. Survival observed in the incident BP cohort was significantly poorer than expected (P<.001). Survival was not different among patients with multisite vs localized disease (P=.90). Limitations Retrospective study design and study population from a small geographic area. Conclusion Incidence of BP in the United States is comparable to that found in Europe and Asia. The mortality rate of BP is lower in the United States than Europe, but higher than previous estimates.

No abstract

This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting.

In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.

No abstract