Abstract

The ichthyoses are a heterogenous group of skin diseases characterized by generalised scaling. Non-bullous Ichthyosiform Erythroderma (NBIE) or congenital ichthyosiform erythroderma is a rare and usually severe autosomal recessive inflammatory ichthyosis. Turner’s syndrome is a condition caused by numeric and structural abnormalities of the X chromosome. In individuals with mosaic Turner’s syndrome, the second X chromosome is not entirely missing. In addition to the expanding list of syndromes that combine with ichthyosis. We report non-bullous ichthyosiform erythroderma in a patient with mosaic Turner’s syndrome. To our knowledge no such association has been reported in literature to date.

Highlights

  • The ichthyoses are a heterogenous group of skin diseases characterized by generalised scaling [1]

  • A chance examination showed that the patient had a short stature and minimal webbing of the neck

  • Since the histopathological features of lamellar ichthyosis and nonbullous ichthyosiform erythroderma are not very accurately distinguishable and clinically the features were in favour, a diagnosis of non-bullous ichthyosiform erythroderma with mosaic form of Turner’s was made

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Summary

Open Access

Non-bullous Ichthyosiform Erythroderma with Mosaic Turner’s Syndrome - a Rare Association.

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