Autoimmune bullous diseases in Austria.

Autoimmune bullous diseases (AIBD) are chronic disorders associated with significant morbidity and even mortality, for which the 19 dermatologic departments in Austria apply standard modalities to provide state-of-the-art diagnosis and treatment. Most of the affected individuals are initially treated on an inpatient basis, with follow-up done in specialized outpatient clinics or in private practices. A well-established system of care for AIBD patients is thus available nationwide. Considering the significant morbidity and mortality but also rareness of AIBD, national and international standardization of AIBD administration in registries is a major requirement of further improvement in patient care.

Epidermolysis bullosa nevi.

Epidermolysis bullosa (EB) nevi are large, eruptive, asymmetrical, often irregularly pigmented melanocytic lesions. Such nevi may give rise to small satellite nevi surrounding the primary nevus, and thus frequently manifest clinical features suggestive of melanoma. They usually arise in sites of previous bullae or erosions. At least twice a year all persisting wounds and EB nevi should be evaluated with a low threshold for histopathologic examination if warranted. Our practice is to punch biopsy EB nevi showing dermoscopic features of concern as well as dermoscopically featureless lesions. Given the skin fragility and potentially impaired wound healing in EB patients, we avoid prophylactic total excision of large EB nevi, but rather use the dermoscope to select appropriate sites for punch biopsies within giant EB nevi.

Diagnostic pitfalls in newborns and babies with blisters and erosions.

Establishing the correct diagnosis in newborns presenting with blisters and erosions is not always a straightforward process. Many different disease entities including acquired (i.e., infectious, immunobullous, traumatic) and inherited disorders have to be taken into consideration. Similarities in clinical appearance, colonization and/or superinfections of preexisting skin lesions, as well as the absence of late changes in the neonate often pose significant diagnostic challenges. In this paper we discuss by giving examples the process of making an accurate diagnosis of blistering skin diseases in the neonatal period on the basis of a diagnostic algorithm. In addition, we provide an overview of the rational use and the limitations of laboratory procedures such as microbial testing, routine light microscopy, immunofluorescence antigen mapping, transmission electron microscopy, and molecular genetic analysis.