DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20211714

Keratolytic Winter Erythema: a misdiagnosed palmoplantar dermatosis

Vidya Kharkar, Anmol Bhargava

Abstract


Keratolytic Winter Erythema, also known as ‘Erythrokeratolysis hiemalis’ or ‘Oudtshoorn disease’ is a rare genetic disorder of keratinization of an unknown etiology characterized by cyclical erythema and intermittent skin peeling usually over palms and soles, particularly during winter.It was originally described in South African families of European descent originating from the Oudtshoorn district of Cape Provence, and has been since identified in several other countries. It is an autosomal dominant disorder with variable penetrance. The condition is often misdiagnosed as Keratolysis exfoliativa, but presence of a relevant family history, winter exacerbation and preceding hyperhidrosis with interdigital involvement differentiate it from the former. We present a case report with clinical histopathological data of an 18-year-old female with attributes of erthrokeratolysis hiemalis. This case is being reported due to its rarity.


Keywords


Keratolytic winter erythema, Oudtshoorn disease, Erythrokeratolysis hiemalis

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References


Starfield M, Hennies HC, Jung M, Jenkins T, Wienker T, Hull P, et al. Localization of the gene causing keratolytic winter erythema to chromosome 8p22-p23, and evidence for a founder effect in South African Afrikaans-speakers. Am J Hum Genet. 1997 Aug;61(2):370-8.

Krahl D, Sigwart A, Hartschuh W, Anton-Lamprecht I, Petzoldt D. Erythrokeratolysis hiemalis. Erythematosquamous genetic dermatosis with seasonal manifestation. Hautarzt. 1994 Nov;45(11):776-9.

Ramsay M, Ngcungcu T, Grayson W. Keratolytic Winter Erythema: An Update. Dermatopathology (Basel). 2019 Jun 26;6(2):126-32.

Findlay GH, Nurse GT, Heyl T, Hull PR, Jenkins T, Klevansky H, et al. Keratolytic winter erythema or 'oudtshoorn skin': a newly recognized inherited dermatosis prevalent in South Africa. S Afr Med J. 1977 Nov 19;52(22):871-4.

Ngcungcu T, Oti M, Sitek JC, Haukanes BI, Linghu B, Bruccoleri R, et al. Duplicated Enhancer Region Increases Expression of CTSB and Segregates with Keratolytic Winter Erythema in South African and Norwegian Families. Am J Hum Genet. 2017 May 4;100(5):737-50.

Oti M, Falck J, Huynen MA, Zhou H. CTCF-mediated chromatin loops enclose inducible gene regulatory domains. BMC Genomics. 2016 Mar 22;17:252.