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Keratolytic winter erythema
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Keratolytic winter erythema : ウィキペディア英語版
Keratolytic winter erythema

Keratolytic Winter erythema (KWE), also known as erythrokeratolysis hiemalis,〔 Oudtshoorn disease and Oudtshoorn skin, is a rare autosomal dominant skin disease of unknown etiology which causes erythema (redness) and keratolysis (peeling) of the skin on the palms and soles. Onset, increased prominence and severity usually occurs during winter. It is a type of genodermatosis.
The name "Oudtshoorn skin" derives from the town of Oudtshoorn in the Western Cape province of South Africa, where the disorder was first described.〔〔 It is one of several genetic disorders known to be highly prevalent among the Afrikaner population.
== Characteristics ==
KWE is characterized by a number of anomalies affecting the skin. Erythema causes redness of the skin, which is generally associated with inflammation and irritation. Including erythema and hyperkeratosis (thickening of the stratum corneum),〔 naturally occurring keratolytic peeling and scaling, with increased manifestation in winter, are prevailing features of the disorder.〔〔
Erythema in KWE has been attributed to necrobiosis (cellular death) within the malpighian layer (the innermost layer of the epidermis).〔 Peeling and scaling are caused by spreading dissection of the stratum corneum, correlating to the underlying necrobiosis.〔
The effects of KWE appear intermittently as patches on the skin of the palms and soles, with these patches appearing on the limbs, buttocks and torso in severe cases.〔〔〔 Facial lesions of this type have also been reported with the disorder, though this is considered to be an extremely rare occurrence.〔
Onset and cyclical recurrence of KWE have shown to be associated with the arrival of winter, or winter-like weather.〔〔 Worsening of symptoms during this time may be considered as an indicator of recurrent onset in patients known to have the disorder, and age of initial onset can be from early childhood to young adulthood, with attenuation of symptoms sometimes happening after age 30.〔〔 Patients first exhibiting the disorder at a younger age may also experience worsened symptoms.〔 Currently, no specific correlating factor or reason for winter-related manifestation has been established, though the coldness and dryer air common to winter conditions may be suspect.〔 Winter onset is, however, considered to be a distinguishing feature of KWE among other erythematic skin disorders.〔
When peeling of skin occurs, the newly exposed layer of skin underneath is moist, raw and very sensitive. While this may result in minor discomfort and inconvenience, in severe cases of KWE where large areas of raw skin are present, it is often life-altering and debilitating.〔

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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