Abstract
Ichthyosis fetalis is a very rare and life‐threatening dermatological disorder that is very difficult to treat, especially in low‐resource settings.
Keywords: congenital, harlequin, ichthyosis, neonate
Ichthyosis fetalis is a very rare and life‐threatening dermatological disorder that is very difficult to treat, especially in low‐resource settings.
1. 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. 1 , 2 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.
ACKNOWLEDGEMENTS
We would like to acknowledge the baby's parents and the neonatal intensive care unit staffs for they actively supported the process of datacollection and follows up updates of the neonate.
Sereke SG, Berhe SE, Bongomin F. Harlequin fetus: A mayhem in a consanguineous marriage? Clin Case Rep. 2021;9:e04540. 10.1002/ccr3.4540
Funding information
None
DATA AVAILABILITY STATEMENT
The information used and/or analyzed during this clinical image is available from the corresponding author on reasonable request.
REFERENCES
- 1. Hovnanian A. Harlequin ichthyosis unmasked: a defect of lipid transport. J Clin Invest. 2005;115(7):1708‐1710. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2. Liang Q, Xiong F, Liang X, et al. Two successive cases of fetal harlequin ichthyosis: a case report. Exp Ther Med. 2019;17(1):449‐452. [DOI] [PMC free article] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
The information used and/or analyzed during this clinical image is available from the corresponding author on reasonable request.