Keratolytic winter erythema Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 30.12.2021

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Erythrokeratolysis hiemalis; Keratolytic winter erythema; MIM 148370; Oudtshoorn Disease

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Autosomal dominant inherited recurrent erythematous palmoplantar keratosis found in Dutch-born South Africans, but also in a Norwegian family (Ngcungcu T et al. 2017).

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Detectable "tandem duplications" in a non-coding genomic region (8p22-p23) that contains an active enhancer element for CTSB. This leads to upregulation of cathepsin B in the stratum granulosum and subsequent epidermal sloughing (Ramsay M et al. 2019).

Catepsin B is a cysteine protease that plays a significant role in keratinocyte homeostasis. Immunohistochemistry demonstrated increased expression of CTSB, the gene encoding cathepsin B, in lesional skin of KWE; furthermore, immunohistochemistry showed increased reactivity for cathepsin B in the stratum granulosum.

Clinical features
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Mild scaling palmoplantar keratosis (skin peeling) with underlying erythema and traumatic blister formation, which worsens especially in the cold season. The ichthyotic changes can also spread to the forearms and lower legs in the form of figured erythematous scaly ruffs.

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  1. Danielsen AG et al (2001) Erythrokeratolysis hiemalis (keratolytic winter erythema): a case report from Denmark. J Eur Acad Dermatol Venereol 15:255-256.
  2. Findlay GH et al (1978) Erythrokeratolysis hiemalis--keratolytic winter erythema or 'Oudtshoorn skin'.
    Anew epidermal genodermatosis with its histological features. Br J Dermatol 98:491-495.
  3. Ngcungcu T et al. (2017) Duplicated enhancer region Increases Expression of CTSB and Segregates with Keratolytic Winter Erythema in South African and Norwegian Families. Am J Hum Genet 100:737-750.
  4. Ramsay M et al (2019) Keratolytic Winter Erythema: An Update. Dermatopathology (Basel) 6:126-132.

Incoming links (2)

Cathepsin; Cathepsin b;

Outgoing links (1)

Cathepsin b;


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Last updated on: 30.12.2021