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Ichthyosis, Trichothiodystrophy

Abstract

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NORD is very grateful to the members of the Medical and Scientific Advisory Board of the Foundation for Ichthyosis & Related Skin Types for assistance in the preparation of this report.

Synonyms of Ichthyosis, Trichothiodystrophy

IBIDS Syndrome
PIBIDS Syndrome
Tay Syndrome
TTD

Disorder Subdivisions

No subdivisions found.

General Discussion

Trichothiodystrophy is a hereditary disorder characterized by brittle hair, which may be accompanied by a variety of other manifestations. It is sometimes called PIBIDS, a term that refers to the association of Photosensitivity Ichthyosis, Brittle hair, Intellectual impairment, Decreased fertility, and Short stature. Without photosensitivity the condition has been termed IBIDS, and without ichthyosis, BIDS. Many patients have recurrent infections, and abnormalities of the bone and teeth may also occur.

The defining feature of trichothiodystrophy is brittle hair, which is sulfur deficient and, when examined with a microscope and polarized light, demonstrates a characteristic light and dark (tiger tail) banding.

Organizations related to Ichthyosis, Trichothiodystrophy

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