Work‐related skin diseases (WSD) are caused or worsened by a professional activity. Occupational skin diseases (OSD) need to fulfil additional legal criteria which differ from country to country. OSD range amongst the five most frequently notified occupational diseases (musculoskeletal diseases, neurologic diseases, lung diseases, diseases of the sensory organs, skin diseases) in Europe.

Just over 25 years ago, De Weerdt and Castelain [1] described the combination of progressive muscular weakness and dystrophic epidermolysis bullosa with an autosomal recessive pattern. Niemi et al. [2] described a Finnish sibship with normal parents and 12 members. Two male sibs died early from severe skin disease but two others survived to adulthood with a combination of severe epidermolysis bullosa and limb girdle muscular dystrophy. The possibility of coincidental recessive disorders was raised but further reports [3,4,5] favoured a specific syndrome.

Toxic epidermal necrolysis (TEN) is an uncommon condition (Figure 1) with an estimated prevalence of 1.2/million each year. This life-threatening condition is frequently drug-induced (Figure 2) and is characterised by epidermal erythema and inflammation, superficial necrosis, and skin erosions resulting in the skin being shed in large sheets [1]. Infection and metabolic failure are invariable consequences and mortality can approach 70% with an average of 30% in reported cases [2].

Journal of the European Academy of Dermatology and VenereologyVolume 7, Issue 1 p. 91-92 1995 Annual Sofia Dermatological Days November 2–4 1995, Sofia, Bulgaria Dr. J. Pace, Dr. J. Pace Medical School University of Malta Guardamanga MaltaSearch for more papers by this author Dr. J. Pace, Dr. J. Pace Medical School University of Malta Guardamanga MaltaSearch for more papers by this author First published: July 1996 https://doi.org/10.1111/j.1468-3083.1996.tb00571.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Volume7, Issue1July 1996Pages 91-92 RelatedInformation