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Quoted phrase not found in phrase index: "Erythrokeratodermia variabilis et progressiva 5"
Page 1
Retinoids in disorders of keratinization: their use in adults.
Dermatologica. 1987;175 Suppl 1:107-24. doi: 10.1159/000248867.
Dermatologica. 1987.
PMID: 2961628
Review.
Good or excellent results have been seen in other forms of palmoplantar keratoderma including mal de Meleda, Papillon-Lefevre syndrome, erythrokeratodermia variabilis, verrucous epidermal nevi, Darier disease and pityriasis rubra pilaris. In patients with Darier dis …
Good or excellent results have been seen in other forms of palmoplantar keratoderma including mal de Meleda, Papillon-Lefevre syndrome, e …
Sphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia.
Bariana TK, Labarque V, Heremans J, Thys C, De Reys M, Greene D, Jenkins B, Grassi L, Seyres D, Burden F, Whitehorn D, Shamardina O, Papadia S, Gomez K, BioResource N, Van Geet C, Koulman A, Ouwehand WH, Ghevaert C, Frontini M, Turro E, Freson K.
Bariana TK, et al.
Haematologica. 2019 May;104(5):1036-1045. doi: 10.3324/haematol.2018.204784. Epub 2018 Nov 22.
Haematologica. 2019.
PMID: 30467204
Free PMC article.
KDSR or 3-keto-dihydrosphingosine reductase is an essential enzyme for de novo sphingolipid synthesis, and pathogenic mutations in KDSR result in the severe skin disorder erythrokeratodermia variabilis et progressiva-4 Four of the eight reported cases …
KDSR or 3-keto-dihydrosphingosine reductase is an essential enzyme for de novo sphingolipid synthesis, and pathogenic mutations in KDSR resu …
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Erythrokeratodermia variabilis in a patient followed through the first year of life.
Bond MJ, Donelan P, Fenske N.
Bond MJ, et al.
Cutis. 1982 Nov;30(5):633-7.
Cutis. 1982.
PMID: 7172743
Erythrokeratodermia variabilis (EKV) is a well-described entity consisting of transient erythematous areas and fixed hyperkeratotic plaques. ...
Erythrokeratodermia variabilis (EKV) is a well-described entity consisting of transient erythematous areas and fixed hyperkera …
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