keratolytic winter erythema
Definition An autosomal dominant (OMIM:1483790) dermatosis traceable to certain 19th-century inhabitants of the province of Oudtshoorn, South Africa, with striking exacerbation in cold weather, starting in March or April and continuing until August to October.
Clinical findings Intermittent and recurrent centrifugal skin peelin with palmoplantar erythaema which in more severe cases, extends up the limbs to the buttocks and trunk. In most patients the inconvenience is moderate; in some it is incapacitating.
Affected ages The age of onset varies from infancy to early adulthood. It tends to to subside in intensity after age 30.
Molecular pathology The genetic locus for KWE is thought to lie on chromosome 8p23-p22.
Synonym Oudtshoorn skin disease