Hailey-Hailey, syndrome
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(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