Harlequin ichthyosis

Harlequin ichthyosis (HI) is the most severe variant of autosomal recessive congenital ichthyosis (ARCI; see this term). It is characterized at birth by the presence of large, thick, plate-like scales over the whole body associated with severe ectropion, eclabium, and flattened ears, that later develops into a severe scaling erythroderma.

Eclabion

A turning outward of the lip or lips, that is, eversion of the lips.


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PMID (PMCID)
27267339
 FEMALE
Generalized morphea in a child with harlequin ichthyosis: a rare association.
Giacomin MF, Franca CM, Oliveira ZN, Machado MC, Sallum AM, Silva CA.
Rev Bras Reumatol Engl Ed. 2016;56(1):82-5.
Harlequin ichthyosis (HI) is a severe and rare hereditary congenital skin disorder characterized by excessive dryness, ectropion and eclabion.