Two cases of lamellar ichthyosis

( views:397, downloads:0 )
Author:
AO Jun-hong(Department of Dermatology, General Hospital of Beijing Military Region of PLA, Beijing 100700, China)
CONG Lin(Department of Dermatology, General Hospital of Beijing Military Region of PLA, Beijing 100700, China)
HAO Zhen-feng(Department of Dermatology, General Hospital of Beijing Military Region of PLA, Beijing 100700, China)
YANG Rong-ya(Department of Dermatology, General Hospital of Beijing Military Region of PLA, Beijing 100700, China)
Journal Title:
INTERNATIONAL JOURNAL OF DERMATOLOGY AND VENEREOLOGY
Issue:
Volume 36, Issue 05, 2010
DOI:
10.3760/cma.j.issn.1673-4173.2010.05.001
Key Word:
Ichthyosis, lamellar;Case reports

Abstract: Two patients presented with skin lesions characterized by beige or grayish brown, polygonal,rhombic or sheeted scales over the body. The scales were adherent in the center, free and elevated at the edges. Some scales were thick as armor. Ectropion palpebrarum, eclabium and hyperkeratosis of palms and soles were observed in both patients. Histopathology of lesions in patient 2 revealed hyperkeratosis,hypergranulosis, acanthosis, elongation of trochanterellus, and small inflammatory infiltrates around blood vessels in upper dermis. Symptoms were relieved in both patients after treatment with oral tretinoin, topical urea cream and cod liver oil ointment.

  • [1]Akiyama M,Sawamura D,Shimizu H.The clinical spectrum of nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis.Clin Exp Dermatol,2003,28(3):235-240.
  • [2]Elias PM,Crumrine D,Rassner U,et al.Basis for abnormal desquamation and permeability barrier dysfunction in RXLI.J Invest Dermatol,2004,122(2):314-319.
  • [3]Fischer J,Faure A,Bouadjar B,et al.Two new loci for autosomal recessive ichthyosis on chromosomes 3p21 and 19p12-q12 and evidence for further genetic heterogeneity.Am J Hum Genet,2000,66(3):904-913.
  • [4]Krebsová A,Küster W,Lestringant GG,et al.Identification,by homozygosity mapping,of a novel locus for autosomal recessive congenital ichthyosis on chromosome 17p,and evidence for further genetic heterogeneity.Am J Hum Genet,2001,69(1):216-222.
  • [5]Nemes Z,Marekov LN,Fésüs L,et al.A novel function for transglutaminase 1:attachment of long-chain omega-hydroxyceramides to involucrin by ester bond formation.Proc Natl Acad Sci U S A,1999,96(15):8402-8407.
  • [6]Jobard F,Lefèvre C,Karaduman A,et al.Lipoxygenase-3(ALOXE3) and 12(R)-lipoxygenase (ALOX12B) are mutated in non-bullous congenital ichthyosiform erythroderma (NCIE) linked to chromosome 17p13.1.Hum Mol Genet,2002,11 (1):107-113.
  • [7]Haenssle HA,Finkenrath A,Hausser I,et al.Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis.Clin Exp Dermatol,2008,33(5):578-581.
WanfangData CO.,Ltd All Rights Reserved
About WanfangData | Contact US
Healthcare Department, Fuxing Road NO.15, Haidian District Beijing, 100038 P.R.China
Tel:+86-010-58882616 Fax:+86-010-58882615 Email:yiyao@wanfangdata.com.cn