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Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome.
J Dermatol 2014; 41(5):371-6JD

Abstract

Keratosis pilaris and ulerythema ophryogenes (keratosis pilaris atrophicans faciei) are part of a group of hereditary disorders of hair follicle keratinization involving follicular inflammation and subsequent atrophy. Monosomy 18p refers to a chromosomal disorder resulting from the deletion of all or part of the short arm of chromosome 18. This trias was first described in a patient by Zouboulis et al. (1994) and has been reported by different authors in four additional patients since then. We have reviewed the five almost identical cases that have been reported in 20 years and we suggest the existence of a new rare syndrome characterized by the trias keratosis pilaris, ulerythema ophryogenes and monosomy 18p. Recognition of the syndrome could assist in early diagnosis of monosomy 18p in these patients.

Authors+Show Affiliations

Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, Germany.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

24801913

Citation

Liakou, Aikaterini I., et al. "Trias of Keratosis Pilaris, Ulerythema Ophryogenes and 18p Monosomy: Zouboulis Syndrome." The Journal of Dermatology, vol. 41, no. 5, 2014, pp. 371-6.
Liakou AI, Esteves de Carvalho AV, Nazarenko LP. Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome. J Dermatol. 2014;41(5):371-6.
Liakou, A. I., Esteves de Carvalho, A. V., & Nazarenko, L. P. (2014). Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome. The Journal of Dermatology, 41(5), pp. 371-6. doi:10.1111/1346-8138.12442.
Liakou AI, Esteves de Carvalho AV, Nazarenko LP. Trias of Keratosis Pilaris, Ulerythema Ophryogenes and 18p Monosomy: Zouboulis Syndrome. J Dermatol. 2014;41(5):371-6. PubMed PMID: 24801913.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome. AU - Liakou,Aikaterini I, AU - Esteves de Carvalho,André V, AU - Nazarenko,Lujdmila P, PY - 2013/11/18/received PY - 2014/01/12/accepted PY - 2014/5/8/entrez PY - 2014/5/8/pubmed PY - 2015/5/16/medline KW - 18p monosomy KW - Zouboulis syndrome KW - genodermatosis KW - hair follicle KW - keratosis pilaris KW - ulerythema SP - 371 EP - 6 JF - The Journal of dermatology JO - J. Dermatol. VL - 41 IS - 5 N2 - Keratosis pilaris and ulerythema ophryogenes (keratosis pilaris atrophicans faciei) are part of a group of hereditary disorders of hair follicle keratinization involving follicular inflammation and subsequent atrophy. Monosomy 18p refers to a chromosomal disorder resulting from the deletion of all or part of the short arm of chromosome 18. This trias was first described in a patient by Zouboulis et al. (1994) and has been reported by different authors in four additional patients since then. We have reviewed the five almost identical cases that have been reported in 20 years and we suggest the existence of a new rare syndrome characterized by the trias keratosis pilaris, ulerythema ophryogenes and monosomy 18p. Recognition of the syndrome could assist in early diagnosis of monosomy 18p in these patients. SN - 1346-8138 UR - https://www.unboundmedicine.com/medline/citation/24801913/Trias_of_keratosis_pilaris_ulerythema_ophryogenes_and_18p_monosomy:_Zouboulis_syndrome_ L2 - https://doi.org/10.1111/1346-8138.12442 DB - PRIME DP - Unbound Medicine ER -