Cover Image

Annular Variant of Epidermolytic Ichthyosis - A Rare Case Report

Kaviya M, Aravamuthan Ramesh. .

Abstract


Bullous congenital ichthyosiformerythroderma of Brocq or epidermolyticichthyosis is a rare mechanobullous eruption first described by Brocq in 1902. This autosomal dominant disorder is characterized by blistering and erythroderma in earlylife due to keratin K1 or K10 mutations withsubsequent development ofichthyosis and       hyperkeratosis. Epidermolytic hyperkeratosis is the hallmark feature on light and electron microscopy. Annular                 epidermolyticichthyosis is a variant of epidermolyticichthyosis that is uncommon in occurrence.

     Here we present a case report of 23 year old male with annular variant of epidermolyticichthyosis over trunks and flexures.

 Keywords: Epidermolyticichthyosis, annular variant


Full Text:

PDF

References


Sahn EE, et al. Annular epidermolyticichthyosis: A unique phenotype. J Am AcadDermatol 1992;27: 348-55

Jha A, Taneja J, Ramesh V, Singh A. Annular epidermolyticichthyosis: A rare phenotypic variant of bullous congenital ichthyosiformerythroderma. Indian J DermatolVenereolLeprol 2015;81:194-7.

Sybert VP, et al. Cyclic ichthyosis with epidermolytic hyperkeratosis: A phenotype conferred by mutations in the 2B domain of keratin k1. Am J Hum Genet 1999;64:732-8

Joh G-Y, et al. A novel dinucleotide mutation in keratin 10 in the annular epidermolyticichthyois variant of bullous congenital ichthyosiserythroderma. J Invest Dermatol 1997;108:357-61

Naik NS. Annular epidermolyticichthyosis. Dermatol Online J 2003;9:4

Timir Y. Mehta, Neela V.Bhuptani, Pratik B. Sheth. Disorders of keratinization. In: Sacchidanand.S, Oberai Chetan, Inamadar Arun.C, editors. IADVL Textbook of dermatology.4th ed.vol.1. Mumbai: Bhalani; 2015. P.361-363

VinzenzOji, DieterMetze and Heiko Traupe. Inherited Disorders of cornification. In: Griffiths C.E.M, Barker B, Bleiker T, Chalmers R, Creamer D, editors. Rooks Textbook of Dermatology. 9th ed.vol.2.UK: John Wiley & Sons; 2016. P.65.13-15.


Refbacks

  • There are currently no refbacks.


Creative Commons License
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.

An initiative of The Tamil Nadu Dr M.G.R. Medical University