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Calabresi V.,Molecular and Cell Biology Laboratory | Sinistro A.,Molecular and Cell Biology Laboratory | Cozzani E.,DISSAL Section of Dermatology | Cerasaro C.,Molecular and Cell Biology Laboratory | And 6 more authors.
Journal of the European Academy of Dermatology and Venereology | Year: 2014

Background: Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease characterized by tissue-bound and circulating autoantibodies to the dermal-epidermal junction. The autoantibody target is type VII collagen (Col VII) which is involved in dermal-epidermal adhesion. Diagnosis is made by clinical and histopathological findings, linear deposition of autoantibodies at the dermal-epidermal junction detected by direct immunofluorescence, and binding to the dermal side of salt-split skin by indirect immunofluorescence (IIF). However, the detection of specific anti-Col VII reactivity has an important confirmatory value. Methods: The humoral immune response in EBA sera was analysed by (i) IIF on human skin, (ii) a commercial Col VII ELISA, and (iii) immunoblotting on Col VII produced by an epithelial cell line. Objective: The aim of this study was to compare the sensitivity of different approaches for the serological diagnosis of EBA. Results: The vast majority of EBA sera (79.2%) bound to the Col VII non-collagenous domains by a commercial ELISA, while a small proportion of patients (12.5%) exclusively reacted to the collagenous domain by immunoblotting. Of note, the autoantibodies reactivity to Col VII was more frequently detected by IB (91.7%) than by IIF (83.3%) and ELISA (79.2%). Interestingly, 2 out of 24 sera recognized Col VII epitopes undetectable in the native secreted protein but present in the context of extracellular matrix proteins, as assessed by immunomapping on Col VII-deficient skin. Conclusion: Our findings show that the use of multiple assays allows to improve diagnostic performance. An algorithm for efficient serological diagnosis of EBA is proposed. © 2013 European Academy of Dermatology and Venereology. Source

Cozzani E.,DISSAL Section of Dermatology | Gasparini G.,DISSAL Section of Dermatology | Burlando M.,DISSAL Section of Dermatology | Drago F.,DISSAL Section of Dermatology | Parodi A.,DISSAL Section of Dermatology
Autoimmunity Reviews | Year: 2015

Bullous pemphigoid may occur in extremely variegated manners, misleading even experienced dermatologists. Indeed the type and/or distribution of lesions may be unusual. Furthermore, there may be an atypical demographic profile of patients, a different clinical course and a different responsiveness to therapy. Up to 20% of the cases the onset is characterized by a non-bullous phase, lasting weeks, months or in particular cases remaining the only manifestation of the disease. During this early phase lesions are generally pruritic erythematous, eczematous or urticarial; however, lesions may also resemble polycyclic, targetoid, nodular or lichenoid lesions. These atypical lesions may also coexist with typical bullae. Other atypical presentations include a vesicular eruption and an erythroderma. Manifestations in children differ from adult forms, presenting an exclusive genital involvement in 50% of cases or a preponderant involvement of the face, the palms and the soles. Rarely bullous pemphigoid is confined to certain body areas, due to particular triggering factors or to a lower disease activity. Therefore, the need to formulate universally recognized diagnostic criteria is increasingly evident, especially for atypical bullous pemphigoid. Direct immunofluorescence of perilesional skin and detection of circulating autoantibodies are mandatory in the diagnosis, especially when the clinical presentation is doubtful. © 2015 Elsevier B.V. Source

Drago F.,DISSAL Section of Dermatology | Gasparini G.,DISSAL Section of Dermatology | Signori A.,DISSAL Section of Dermatology | Campisi C.,DISSAL Section of Dermatology | And 2 more authors.
Journal of the European Academy of Dermatology and Venereology | Year: 2015

Abstract Background Dermatological emergencies exist and should not be underestimated. On the other hand, many accesses to the emergency department (ED) of patients with dermatological conditions are unjustified. Objective Our main objective is to describe dermatological conditions seen in an ED observation unit (EDOU). Secondly, our aim is to identify alarm symptoms and signs of 'true emergencies'. Methods We conducted a prospective study, including dermatological patients admitted to EDOU of the University Hospital of San Martino, Genoa, Italy, in 3 years. Results Overall 372 patients were studied. The most common condition seen was infection (41.67%) (mainly bacterial), followed by atypical exanthem (13.98%) and vasculitis (11.29%). The highest rate of cases peaked in May (13%); infectious diseases showed two peaks (spring and autumn). Conclusion Given constraints involved in health care today, it is crucial to understand which dermatological presentations are 'true emergencies'. In such a scenario knowing the epidemiology of dermatological emergencies and the alarming skin signs and symptoms might be useful. © 2014 European Academy of Dermatology and Venereology. Source

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