Ichthyosis bullosa of Siemens

Definition:

Ichthyosis Bullosa of Siemens (IBS) is an autosomal dominant disorder characterized by mild hyperkeratosis and blister formation. The blistering is superficial, and areas of peeling of the skin are known as the 'Mauserung phenomenon'. IBS shows similar features to those in epidermolytic hyperkeratosis, but can be distinguished by the absence of erythema.

References:

[1]. M Akiyama, et al. Ichthyosis bullosa of Siemens: its correct diagnosis facilitated by molecular genetic testing. Br J Dermatol. 2005 Jun;152(6):1353-6. [Content Brief]

[2]. T Basarab, et al. Ichthyosis bullosa of Siemens: report of a family with evidence of a keratin 2e mutation, and a review of the literature. Br J Dermatol. 1999 Apr;140(4):689-95. [Content Brief]

[3]. Y Suga, et al. Hot spot mutations in keratin 2e suggest a correlation between genotype and phenotype in patients with ichthyosis bullosa of Siemens. Exp Dermatol. 2000 Feb;9(1):11-5. [Content Brief]

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