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