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Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, manifests at birth or late in life and is characterised by intense pruritus, resulting in lichenified or nodular prurigo-like lesions and scarring most prominent on the shins. Treatment is unsatisfactory. We report a patient treated with success using a combination of topical and systemic agents.

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Chemical and physical characterization of transported evolving aerosols in an in vitro system is complex. The challenges include appropriate sampling sensitivity, measurement capabilities, and performing online measurements of constituents in the flowing aerosol during exposure. We assessed the performance of single-photon ionization mass spectrometry in measuring aerosol properties within an in vitro aerosol exposure system. The sampling efficiency of the instrument was studied under three protocols to capture the evolving aerosol process inside the exposure system, and it was evaluated using computational fluid dynamics modeling. The changes in the aerosol as dilution is applied show not only a reduction in concentration of the traced substances but also selective sampling due to evolution of the aerosol and (gas/liquid) phase partitioning of the substances forming the aerosol or a change in the aerosol properties. These effects have potentially a direct impact on the delivered dose, as aerosol deposition is dependent on particle size. Dilution affects the chemical concentration of the substances as well as the interconnected physical properties of the aerosol; therefore, the experimental design of in vitro studies should not only report the dilution flow rates but also details of the applied dilution protocol. This adds a layer of complexity to the design and comparison of studies. We also discuss the potential and limitations of single-photon ionization mass spectrometry as a tool in in vitro monitoring of aerosols.

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and the jj Liver Unit (and small bowel transplantation), Birmingham's Children Hospital, UK.