Ichthyosis hystrix gravior

Overview

Type of disease: Rare conditions

Ichthyosis hystrix gravior, also known as ichthyosis hystrix, Lambert type, is one form of ichthyosis, an extremely rare genetic syndrome in which dead skin cells accumulate in thick, dry scales on your skin’s surface, thickening of the outer layer of skin and pattern of the horn material points resembling ‘quill-like’ projections or spiny scales which cover the entire body except the face, genitals, palms and soles. Between 1731 and 1851, the Lambert family of Suffolk, England, had 11 members in 4 generations with ichthyosis hystrix gravior, the only known cases to date.

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