Hailey-Hailey, syndrome

(OMIM 169.600)

(benign familial pemphigus)

Rare. Due to an anomaly of the gene which codes for the SPCA1 protein (secretory pathway calcium/manganese ATPase) on 3q21-24 which causes a decreased adhesion between the keratocytes. 

Clinically: lesions at the level of the skin folds following friction of the skin during a movement.


Anesthetic implications: 

avoid movements with skin friction, sweat and excessive skin temperature. Often surinfected lesions. Some of the ointments used contain corticoids (adrenal function ?)


References : 

-         Shah A. 
Anaesthesia in patients with Hailey-Hailey disease. 
Anaesthesia 2006; 61: 514.


Updated: April 2019