Case of bullous pemphigoid in a 28-year-old woman affected by tuberous sclerosis complex

[No abstract available

Una strana placca eritematosa parzialmente ulcerata

Presentiamo il caso di una donna di 38 anni affetta da sclerosi tuberosa ed in terapia immunosoppressiva dal 2006 per trapianto renale. La paziente presentava grave deficit cognitivo e di coordinazione motoria. All’esame obiettivo si rilevava la presenza di una placca eritematosa in regione pretibiale sinistra, parzialmente ulcerata, di cui la paziente riferiva comparsa e persistenza da circa 3 anni. Nel resto del corpo la cute si presentava integra eccetto alcune lesioni da grattamento. In passato erano state eseguite due biopsie cutanee per un miglior inquadramento diagnostico il cui referto istologico era risultato completamente aspecifico. La madre riferiva di aver notato la comparsa talvolta di alcune bolle sul corpo, che si rompevano e guarivano spontaneamente senza lasciare esiti cicatriziali. Nel sospetto di una patologia bollosa si eseguiva un prelievo ematico per immunofluorescenza indiretta e due biopsie cutanee per esame istologico e immunofluorescenza diretta. Al momento della consegna degli esiti degli esami, che deponevano per un quadro di pemfigoide bolloso, si osservava un’intensa area disepitelizzata nella regione pretibiale sinistra e due piccole bolle tese sulla gamba destra. Localmente si applicavano steroide topico sulle lesioni bollose e medicazione a base di idrofibra con argento sull’area pretibiale sinistra con risoluzione del quadro cutaneo

Rethinking neuroprotection in severe traumatic brain injury : toward bedside neuroprotection

Neuroprotection after traumatic brain injury (TBI) is an important goal pursued strenuously in the last 30 years. The acute cerebral injury triggers a cascade of biochemical events that may worsen the integrity, function, and connectivity of the brain cells and decrease the chance of functional recovery. A number of molecules acting against this deleterious cascade have been tested in the experimental setting, often with preliminary encouraging results. Unfortunately, clinical trials using those candidate neuroprotectants molecules have consistently produced disappointing results, highlighting the necessity of improving the research standards. Despite repeated failures in pharmacological neuroprotection, TBI treatment in neurointensive care units has achieved outcome improvement. It is likely that intensive treatment has contributed to this progress offering a different kind of neuroprotection, based on a careful prevention and limitations of intracranial and systemic threats. The natural course of acute brain damage, in fact, is often complicated by additional adverse events, like the development of intracranial hypertension, brain hypoxia, or hypoperfusion. All these events may lead to additional brain damage and worsen outcome. An approach designed for early identification and prompt correction of insults may, therefore, limit brain damage and improve results

Current and emerging technologies for probing molecular signatures of traumatic brain injury

Traumatic brain injury (TBI) is understood as an interplay between the initial injury, subsequent secondary injuries, and a complex host response all of which are highly heterogeneous. An understanding of the underlying biology suggests a number of windows where mechanistically inspired interventions could be targeted. Unfortunately, biologically plausible therapies have to-date failed to translate into clinical practice. While a number of stereotypical pathways are now understood to be involved, current clinical characterization is too crude for it to be possible to characterize the biological phenotype in a truly mechanistically meaningful way. In this review, we examine current and emerging technologies for fuller biochemical characterization by the simultaneous measurement of multiple, diverse biomarkers. We describe how clinically available tech- niques such as cerebral microdialysis can be leveraged to give mechanistic insights into TBI pathobiology and how multiplex proteomic and metabolomic techniques can give a more complete description of the underlying biology. We also describe spatially resolved label-free multiplex techniques capable of probing structural differences in chemical signatures. Finally, we touch on the bioinformatics challenges that result from the acquisition of such large amounts of chemical data in the search for a more mechanistically complete description of the TBI phenotype.GV received a fellowship by “Associazione Amici del Mario Negri.

How to integrate surgery and targeted therapy with biologics for the treatment of hidradenitis suppurativa: Delphi consensus statements from an Italian expert panel

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent and painful nodules and abscesses in intertriginous skin areas, which can progress to sinus tract formation, tissue destruction, and scarring. HS is highly debilitating and severely impairs the psychological well-being and quality of life of patients. The therapeutic approach to HS is based on medical therapy and surgery. First-line medical therapy includes topical antibiotics, systemic antibiotics, and biologics. Main surgical procedures include deroofing, local excision, and wide local excision. Despite the availability of multiple therapeutic options, the rates of disease recurrence and progression continue to be high. In recent years, the possibility of combining biologic therapy and surgery has raised considerable interest. In a clinical trial, the perioperative use of adalimumab has been associated with greater response rates and improved inflammatory load and pain, with no increased risk of postoperative infectious complications. However, several practical aspects of combined biologic therapy and surgery are poorly defined. In June 2022, nine Italian HS experts convened to address issues related to the integration of biologic therapy and surgery in clinical practice. To this purpose, the experts identified ten areas of interest based on published evidence and personal experience: 1) patient profiling (diagnostic criteria, disease severity classification, assessment of response to treatment, patient-reported outcomes, comorbidities); 2) tailoring surgery to HS characteristics; 3) wide local excision; 4) pre-surgery biologic treatment; 5) concomitant biologic and surgical treatments; 6) pre- and post-surgery management; 7) antibiotic systemic therapy; 8) biologic therapy after radical surgery; 9) management of adverse events to biologics; 10) management of postoperative infectious complications. Consensus between experts was reached using the Estimate-Talk-Estimate method (Delphi Method). The statements were subsequently presented to a panel of 27 HS experts from across Italy, and their agreement was assessed using the UCLA Appropriateness Method. This article presents and discusses the consensus statements

Uncertainty estimations for moment tensor inversions: the issue of the 2012 May 20 Emilia earthquake

This article has been accepted for publication in Geophysical Journal Internationa ©: 2016 Published by Oxford University Press on behalf of the Royal Astronomical Society. All rights reserved.Seismic moment tensor is one of the most important source parameters defining the earthquake dimension and style of the activated fault. Geoscientists ordinarily use moment tensor cata- logues, however, few attempts have been done to assess possible impacts of moment magnitude uncertainties upon their analysis. The 2012 May 20 Emilia main shock is a representative event since it is defined in literature with a moment magnitude value (Mw) spanning between 5.63 and 6.12. A variability of ∼0.5 units in magnitude leads to a controversial knowledge of the real size of the event and reveals how the solutions could be poorly constrained. In this work, we investigate the stability of the moment tensor solution for this earthquake, studying the effect of five different 1-D velocity models, the number and the distribution of the stations used in the inversion procedure. We also introduce a 3-D velocity model to account for structural hetero- geneity. We finally estimate the uncertainties associated to the computed focal planes and the obtained Mw. We conclude that our reliable source solutions provide a moment magnitude that ranges from 5.87, 1-D model, to 5.96, 3-D model, reducing the variability of the literature to ∼0.1. We endorse that the estimate of seismic moment from moment tensor solutions, as well as the estimate of the other kinematic source parameters, requires coming out with disclosed assumptions and explicit processing workflows. Finally and, probably more important, when moment tensor solution is used for secondary analyses it has to be combined with the same main boundary conditions (e.g. wave-velocity propagation model) to avoid conflicting results.Published792-8062T. Sorgente SismicaJCR Journa

Una strana placca eritematosa parzialmente ulcerata

Presentiamo il caso di una donna di 38 anni affetta da sclerosi tuberosa ed in terapia immunosoppressiva dal 2006 per trapianto renale. La paziente presentava grave deficit cognitivo e di coordinazione motoria. All’esame obiettivo si rilevava la presenza di una placca eritematosa in regione pretibiale sinistra, parzialmente ulcerata, di cui la paziente riferiva comparsa e persistenza da circa 3 anni. Nel resto del corpo la cute si presentava integra eccetto alcune lesioni da grattamento. In passato erano state eseguite due biopsie cutanee per un miglior inquadramento diagnostico il cui referto istologico era risultato completamente aspecifico. La madre riferiva di aver notato la comparsa talvolta di alcune bolle sul corpo, che si rompevano e guarivano spontaneamente senza lasciare esiti cicatriziali. Nel sospetto di una patologia bollosa si eseguiva un prelievo ematico per immunofluorescenza indiretta e due biopsie cutanee per esame istologico e immunofluorescenza diretta. Al momento della consegna degli esiti degli esami, che deponevano per un quadro di pemfigoide bolloso, si osservava un’intensa area disepitelizzata nella regione pretibiale sinistra e due piccole bolle tese sulla gamba destra. Localmente si applicavano steroide topico sulle lesioni bollose e medicazione a base di idrofibra con argento sull’area pretibiale sinistra con risoluzione del quadro cutaneo