Acute generalized Hailey-Hailey disease

Abstract

A patient with extensive histologically proven Hailey-Hailey disease is described whose initial clinical presentation was suggestive of erythema multiforme or toxic epidermal necrolysis. This potentially misleading morphology of acute proven Hailey-Hailey disease has not been described previously and may be a consequence of bacterial infection exacerbating acantholysis.

Bacterial infection-induced generalized Hailey-Hailey disease successfully treated by etretinate.

“…P. aeruginosa and Staphylococcus species were detected in cultures from the intertriginous plaques of our patient and the superficial infection was thought to act as a trigger for the formation of the generalized HHD lesion. As described in the previous reports, 5,8 superficial cutaneous infection could initiate the exacerbation of HHD through a Koebner‐like response.…”

“…Generalized forms of HHD are remarkably rare events. The exact pathogenetic mechanisms of generalized HHD have not yet been clarified, although bacterial and herpes simplex virus infections, arthropod infestation, and drug eruptions are thought to be possible triggers 3–8 . P. aeruginosa and Staphylococcus species were detected in cultures from the intertriginous plaques of our patient and the superficial infection was thought to act as a trigger for the formation of the generalized HHD lesion.…”

Disseminierter M. Hailey-Hailey

“…B. auf den Brustbereich und den Unterarm. Es existieren Fallbeschreibungen,bei denen disseminierte Hautveränderungen eines M. Hailey-Hailey klinisch unter dem Bild eines Erythema multiforme oder einer toxisch-epidermalen Nekrolyse imponierten [2]. Als mögliche Provokationsfaktoren wurden u.a.chronische Irritation oder Skabies beschrieben, eine erbliche Komponente wurde vermutet [4,9].…”

“…In anderen Studien wurde das Auftreten von Nävi spili bei Neugeborenen nicht beschrieben, sodass die kongenitale Anlage des Nävus spilus angezweifelt wird [2,14]. Auch bezüglich des Managements herrschen noch Unklarheiten.…”

A novel nonsense ATP2C1 mutation causes Hailey-Hailey disease in a Tunisian family

“…The disease has a fluctuating course with remissions and exacerbations [3]. In rare instances, the rash may become generalized and the skin lesions may even develop into squamous cell carcinoma [4]. Penetrance in adults affected by HHD is complete but expressivity is variable [2].…”