Description

The Peeling Skin Syndrome is a rare inherited disorder with a separation of the epidermis within the stratum corneum associated with an abnormal keratin.


 

Inheritance: autosomal recessive

Gene implicated: transglutaminase 5 (TGM5) in some cases

 

Features:

(1) spontaneous onset of skin peeling, sometimes triggered by exposure to water, humidity or sweating

(2) painless and asymptomatic

(3) variable family history

(4) absence of inflammation and bleeding

(5) exclusion of alternative diagnoses

 

Forms:

(1) generalized

(2) localized (acral, other)

 


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