From pemphix to desmogleins Daška Štulhofer Buzina, MD ⁎ , Branka Marinović, MD Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine, University of Zagreb, Salata 4, 10000 Zagreb, Croatia Abstract 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. © 2011 Elsevier Inc. All rights reserved. Origin of the word pemphyx and pemphigus The word pemphigus originates from the Greek word πέμφιξ, ίγος,η, meaning air, breath. The ancient authors usually gave short and incomplete clinical pictures, so it is often difficult to conclude the origin of disease connected with the name pemphigus. Walter Lever and John Talbott, when they were at the Massachusetts General Hospital in Boston, conducted a historical study of pemphigus so that we can follow the progress of the understanding blistering diseases and pemphigus from ancient times to the modern concept of pemphigus. The first mention of pemphigus came from Hippocrates (460-370 BCE) as “pemphigodes pyretoi” when he listed different types of fever in his writings. Giving no further explanation, he described the disease as terrible in its appearance. It seems this was not a bullous disease, because it was not placed with other blistering diseases. All blister eruptions described were of short duration, which gives grounds to the statement that pemphigus was unknown to him. 1 Febris pemphigodes is a disease described by Galen (131-210 CE), characterized by expansion of “phlyc- tides” (in Latin, pustules) in the mouth during fever. The disease was characterized by vesicles in the mouth, probably herpes labialis, as Hebra assumed by reviewing the history of pemphigus in his textbook. 2 Medieval physicians adhered closely to the writings of the great Greek and Roman authorities and rarely added clinical observations of their own. Later, in the Middle Ages, in his book Praxis medicina admiranda, published in 1632, Abraham Zacutus Luisitanus (1575- 1642) used the term pemphigoid fever (de febri pemphigode) for a case report of a blistering disease in a man who had two attacks of unknown duration, so doubt remained whether it was pemphigus. 1 ⁎ Corresponding author. Tel.: +385 1 2368 922; fax: +385 1 2379 911. E-mail address: daska.stulhofer-buzina@zg.htnet.hr (D.Š. Buzina). 0738-081X/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.clindermatol.2011.01.005 Clinics in Dermatology (2011) 29, 355–359