Harlequin fetus: A mayhem in a consanguineous marriage?

Ichthyosis fetalis is a very rare and life-threatening dermatological disorder that is very difficult to treat, especially in low-resource settings.

CASE PRESENTATION

A neonate was born to parents in a consanguineous marriage, with severe form of congenital ichthyosis with characteristic features of a thick, heavily keratinized, and scaly skin all over the body.

Harlequin fetus is a very rare and severe form of congenital ichthyosis characterized by a thick, heavily keratinized, and scaly skin. , A 3.3 kg term male neonate was delivered to a 25‐year‐old Eritrean woman. The baby was covered with thick yellowish to whitish scales, split by extensive some deep and others shallow fissures extending to the dermis. The scales covered the whole body. There was severe ectropion. The scalp hairs were present; the limbs were edematous and inflexible digits due to taut skins (Figure 1A–C). The pregnancy was uneventful. Both parents were paternal first‐degree cousins. The baby was admitted to neonatal intensive care and was put on topical retinoids, intravenous and topical antibiotics, and eye drops. On the second day of life (Figure 1D), neonatal sepsis ensued, and the neonate deteriorated clinically, and parents decided to take their baby home.

FIGURE 1

A, B, and C (day zero) and D (day one). A, B, and C, demonstrated yellowish to whitish scales split by extensive deep and shallow fissures extending to the dermis. Widely open mouth, severe ectropion, and edematous limb with inflexible digits due to taut skins were also demonstrated. D, demonstrated that decrement of the yellowish scale with more exposure of the fissures and drying of the fissures edge

AUTHOR CONTRIBUTIONS

All authors made a significantcontribution to the work reported, whether that is in the conception, studydesign, execution, acquisition of data, analysis and interpretation, or in allthese areas; took part in drafting, revising or critically reviewing thearticle; gave final approval of the version to be published; have agreed on thejournal to which the article has been submitted; and agree to be accountablefor all aspects of the work.

ETHICAL APPROVAL AND CONSENT TO PARTICIPATE

No institutional approval was required to publish the clinical image details. The patient provided a written informed consent to participate in the study.

CONSENT FOR PUBLICATION

The patient provided an informed written consent for this case to be published in a peer‐reviewed journal.