Harlequin fetus

Baden, H., Coldsmith, L., The Structural Proteins of Harlequin Fetus... 

Harlequin fetus Very rare Autosomal recessive Death within two weeks Generalized, large plaque-like scales Ectropion eclabion Molecular defect in keratin... 

Figure 13.6 Woman with lamellar ichthyosis and scarring alopecia Harlequin fetus...

Figure 13.7 Harlequin fetus. Photograph courtesy of Dr. Arnesto Macetelo-Ruiz...

Baden, H. P. and Goldsmith, L. A. (1973). The structural proteins of harlequin fetus Stratum corneum. J. Invest. Dermatol, 61,25... 

Congenital ichthyoses include the nigricans form, the lamellar form, the erythrodermatous form and the harlequin fetus, which differ mainly in severity. The affected newborn is commonly of low weight and erythrodermic and often a parchment-like sheet covers the body. All congenital Ichthyoses are inherited as autosomal recessive traits, except for the nigricans form which is X-linked and affects males only. 

Harlequin fetus represents the most severe degree of this condition, death occurring in utero or shortly after birth. The whole skin is thickened, hyper-keratotic and deeply fissured. The facial features are distorted and the extremities flexed because of extreme cutaneous inelasticity. 

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