Immunoadsorption (IA) has been successfully used in a large variety of autoantibody-mediated disorders. In dermatology, IA is increasingly applied as adjuvant treatment for severe and/or refractory autoimmune bullous diseases. These disorders are characterized by autoantibodies against structural proteins of the skin and/or mucous membranes and include, among others, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid. Autoimmune blistering diseases are associated with a high mortality (pemphigus) or morbidity (bullous pemphigoid) and in particular in pemphigus diseases, treatment is challenging.The pathogenetic role of autoantibodies in most of the immunobullous diseases has been clearly demonstrated, therefore, removal of these autoantibodies is a rational therapeutic approach. IA has been shown to effectively lower the serum autoantibodies and to lead to rapid clinical responses. Most recently, IA has been successfully applied in patients with severe atopic dermatitis and high total serum IgE levels. Here, the different treatment protocols, clinical efficacy, and adverse events are summarized.

The cell stress chaperone heat shock protein 90 (Hsp90) has been implicated in inflammatory responses and its inhibition has proven successful in different mouse models of autoimmune diseases, including epidermolysis bullosa acquisita. Here, we investigated expression levels and secretory responses of Hsp90 in patients with bullous pemphigoid (BP), the most common subepidermal autoimmune blistering skin disease. In comparison to healthy controls, the following observations were made: (i) Hsp90 was highly expressed in the skin of BP patients, whereas its serum levels were decreased and inversely associated with IgG autoantibody levels against the NC16A immunodominant region of the BP180 autoantigen, (ii) in contrast, neither aberrant levels of circulating Hsp90 nor any correlation of this protein with serum autoantibodies was found in a control cohort of autoimmune bullous disease patients with pemphigus vulgaris, (iii) Hsp90 was highly expressed in and restrictedly released from peripheral blood mononuclear cells of BP patients, and (iv) Hsp90 was potently induced in and restrictedly secreted from human keratinocyte (HaCaT) cells by BP serum and isolated anti-BP180 NC16A IgG autoantibodies, respectively. Our results reveal an upregulated Hsp90 expression at the site of inflammation and an autoantibody-mediated dysregulation of the intracellular and extracellular distribution of this chaperone in BP patients. These findings suggest that Hsp90 may play a pathophysiological role and represent a novel potential treatment target in BP.

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Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.What is already known about this topic?• Supportive care is the cornerstone of management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in the acute phase.• There is no consensus or guideline on the best supportive care.

Human scabies is usually transmitted during skin-to-skin contact with persons infested with the ectoparasitic mite Sarcoptes scabiei var. hominis. Scabies is a rising major public health issue worldwide and has recently been designated as a neglected tropical disease by the World Health Organization (WHO). 1 Current guidelines propose a range