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Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p.
Pediatr Dermatol 1994; 11(2):172-5PD

Abstract

We report a 13-year-old boy with deletion of the short arm of chromosome 18 and follicular, partially inflammatory, keratotic papules of the eyebrows, foreskin, and cheeks (ulerythema ophryogenes) as well as the shoulders, upper back, upper arms, and thighs (keratosis pilaris), initially diagnosed as atopic dermatitis. Over 100 patients with this genetic defect have been reported, and the 18p- syndrome is considered one of the most frequently occurring deletion syndromes. However, ulerythema ophryogenes and keratosis pilaris have not been described in any of these patients, although the association of the latter with other genetic abnormalities is well known. Keratosis pilaris is a relatively common genodermatosis of ectodermal origin, frequently occurring with ichthyosis or atopy; concomitance with ulerythema ophryogenes has also been reported. The association of chromosome 18p deletion defect and ulerythema ophryogenes may be helpful in future attempts to localize the gene defect responsible for follicular genokeratoses.

Authors+Show Affiliations

Department of Dermatology, University Medical Center Steglitz, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

8041661

Citation

Zouboulis, C C., et al. "Ulerythema Ophryogenes and Keratosis Pilaris in a Child With Monosomy 18p." Pediatric Dermatology, vol. 11, no. 2, 1994, pp. 172-5.
Zouboulis CC, Stratakis CA, Rinck G, et al. Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. Pediatr Dermatol. 1994;11(2):172-5.
Zouboulis, C. C., Stratakis, C. A., Rinck, G., Wegner, R. D., Gollnick, H., & Orfanos, C. E. (1994). Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. Pediatric Dermatology, 11(2), pp. 172-5.
Zouboulis CC, et al. Ulerythema Ophryogenes and Keratosis Pilaris in a Child With Monosomy 18p. Pediatr Dermatol. 1994;11(2):172-5. PubMed PMID: 8041661.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. AU - Zouboulis,C C, AU - Stratakis,C A, AU - Rinck,G, AU - Wegner,R D, AU - Gollnick,H, AU - Orfanos,C E, PY - 1994/6/1/pubmed PY - 1994/6/1/medline PY - 1994/6/1/entrez SP - 172 EP - 5 JF - Pediatric dermatology JO - Pediatr Dermatol VL - 11 IS - 2 N2 - We report a 13-year-old boy with deletion of the short arm of chromosome 18 and follicular, partially inflammatory, keratotic papules of the eyebrows, foreskin, and cheeks (ulerythema ophryogenes) as well as the shoulders, upper back, upper arms, and thighs (keratosis pilaris), initially diagnosed as atopic dermatitis. Over 100 patients with this genetic defect have been reported, and the 18p- syndrome is considered one of the most frequently occurring deletion syndromes. However, ulerythema ophryogenes and keratosis pilaris have not been described in any of these patients, although the association of the latter with other genetic abnormalities is well known. Keratosis pilaris is a relatively common genodermatosis of ectodermal origin, frequently occurring with ichthyosis or atopy; concomitance with ulerythema ophryogenes has also been reported. The association of chromosome 18p deletion defect and ulerythema ophryogenes may be helpful in future attempts to localize the gene defect responsible for follicular genokeratoses. SN - 0736-8046 UR - https://www.unboundmedicine.com/medline/citation/8041661/Ulerythema_ophryogenes_and_keratosis_pilaris_in_a_child_with_monosomy_18p_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0736-8046&date=1994&volume=11&issue=2&spage=172 DB - PRIME DP - Unbound Medicine ER -