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Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome.

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.

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  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, Germany.

    ,

    Source

    The Journal of dermatology 41:5 2014 May pg 371-6

    MeSH

    Abnormalities, Multiple
    Adolescent
    Adult
    Chromosome Deletion
    Chromosome Disorders
    Chromosomes, Human, Pair 18
    Darier Disease
    Diagnosis, Differential
    Eyebrows
    Female
    Humans
    Laminin
    Male
    Syndrome
    Young Adult

    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 -