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Clin Dermatol. 2011 Jul-Aug;29(4):355-9. doi: 10.1016/j.clindermatol.2011.01.005.

From pemphix to desmogleins.

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Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine, University of Zagreb, Salata 4, 10000 Zagreb, Croatia.


In the not so distant past, the word pemphigus or pemphix was common for describing various diseases characterized by blistering as well as various disorders that do not originate from a blistering pathology. Patients with these conditions were grouped in "other" skin diseases. Step by step, during the past, we were introduced to these severe conditions. First, we learned from sporadic case reports, then new differentiations were reported according to histology, later immunopathology was developed, and now there are discoveries of new molecules. Immense progress with new approaches to therapy has been achieved, but much improvement is still needed. The modern definition of pemphigus undoubtedly represents a group of rare, intraepidermal autoimmune bullous diseases characterized by intraepidermal blisters and circulating autoantibodies desmogleins against the keratinocytes cell surface.

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