A sporadic case of ichthyosis hystrix: A rare entity
Keywords:Ichthyosis hystrix, hyperkeratosis, sporadic
AbstractThe term ichthyosis hystrix encompasses several rare ichthyosiform disorders characterized by massive hyperkeratosis with an appearance like spiny scales. This is a rare form of ichthyosis with autosomal dominant inheritance and a very few cases have been reported. We report a case of ichthyosis hystrix in a 22-year-old male patient with hyperkeratotic, dry, warty, scaly excrescences on most of the body surface in a linear pattern. Palms, soles and nails were spared and family history was negative. The case is being reported in view of the rarity of disease and its sporadic occurrence.
Judge MR, McLean WH, Munro CS. Disorders of keratinization. In: Burns T, Breathnach S, Cox N, Griffith C, editors. Rook's Textbook of Dermatology. 8th ed. Singapore: Wiley-Blackwell; 2010. p. 19.1-122.
Kubo Y, Urano Y, Matsuda R, Ishigami T, Murao K, Arase S et al. Ichthyosis hystrix, Curth-Macklin type: A new sporadic case with a novel mutation of keratin 1. Arch Dermatol. 2011;147:999-1001.
Fleckman P, DiGiovanna JJ. The ichthyoses. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. p. 507-38.
Nayak S, Acharjya B, Mohanty P. Ichthyosis hystrix. Indian Dermatol Online J. 2013;4:47-9.
Brocq L. Erythrodermie congénitale ichthyosiforme avec hyperepidermotrophie. Ann Dermatol Syphiligr. 1902;4:1-31.
Gianotti F. Congenital ichthyosiform dermatoses in childhood. J Dermatol (Japan). 1980;7:1-9.
Wang WH, Li LF, Zhang Q, Yang SM, Jiang W, Wang YY et al. Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type. Br J Dermatol. 2007;156:1027-31.
Ishida-Yamamoto A, Richard G, Takahashi H, Iizuka H. In vivo studies of mutant keratin 1 in ichthyosis hystrix Curth-Macklin. J Invest Dermatol. 2003;120:498-500.
Lee JR, White TW. Connexin-26 mutations in deafness and skin disease. Expert Rev Mol Med. 2009;11:e35.