Conradi-Hünermann-Happle syndrome: a novel heterozygous missense mutation, c.204G>T (p.W68C)

Pediatr Dermatol. 2014 Jul-Aug;31(4):493-6. doi: 10.1111/pde.12336. Epub 2014 Jun 11.

Abstract

Conradi-Hünermann-Happle syndrome (X-linked dominant chondrodysplasia punctata, CDPX2 [Online Mendelian Inheritance in Man 302960]) is a rare genodermatosis that presents with blaschkolinear ichthyosis, cicatricial alopecia, chondrodysplasia punctata, asymmetric shortening of the bones, and cataracts. In this case report we describe a child presenting with a patterned alopecia in which supplementary signs and clinical examination of the mother led to the suspicion of Conradi-Hünermann-Happle syndrome. Mutation analysis revealed a heterozygous novel missense mutation, c.204G>T (p.W68C), in exon 2.

Publication types

  • Case Reports

MeSH terms

  • Alopecia / genetics*
  • Chondrodysplasia Punctata / diagnosis
  • Chondrodysplasia Punctata / genetics*
  • Exons / genetics
  • Female
  • Heterozygote
  • Humans
  • Infant
  • Mutation, Missense*
  • Steroid Isomerases / genetics*

Substances

  • Steroid Isomerases
  • EBP protein, human