A Systematic Review of the Association of Elastosis Perforans Serpiginosa and Congenital Disorders
Main Article Content
Keywords
Down syndrome, Ehlers-Danlos syndrome, Elastosis Perforans Serpiginosa, Marfan syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum
Abstract
Background: Elastosis perforans serpiginosa (EPS) is a skin condition marked by transepidermal elimination of abnormal elastic fibers, with a classical presentation of papules or plaques arranged in serpiginous, annular, or arcuate patterns on the neck, face, arms, or other flexural regions. A previous review of the literature reported that approximately 1/4 of EPS cases are associated with congenital disorders, including Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum. To the knowledge of these authors, no review examining the association of EPS and congenital disorders has been performed since 1968.
Objective: The primary objective of this paper is to perform an updated review of the literature focused on the associations between EPS and congenital disorders.
Methods: We searched electronic databases (Medline, Web of Science, PubMed) for literature pertaining to EPS and Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum.
Results: Evidence for the association of EPS and congenital disorders is 57 cases from 48 published papers.
Conclusions: Our results suggest an association between EPS and congenital disorders. However, current evidence is limited to case reports, underscoring the need for future research investigating the relationship between EPS and congenital disorders.
References
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