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- Title
Treatment of extensive elastosis perforans serpiginosa with acitretin in a man with Down syndrome.
- Authors
Enos, Tyler; Vasquez, Rebecca; Vandergriff, Travis; Wang, Richard; Rainwater, Yevgeniya
- Abstract
Elastosis perforans serpiginosa (EPS) is a rare condition, commonly grouped with other perforating dermatoses, in which dermal elastic fibers are transepidermally extruded.1 It is classically described on the head, neck, and flexural sites and is composed of keratotic papules arranged in annular and arcuate arrays.1,2 We report a case of extensive EPS in a man with Down syndrome, successfully treated with acitretin, a second-generation oral retinoid. Additionally, we acknowledge that in patients with Wilson's disease who are taking penicillamine and develop EPS, acitretin should be used with caution given the potential for hepatotoxicity. While EPS may occur in otherwise healthy individuals, many cases manifest in patients with underlying genetic or acquired disorders, including: acrogeria, Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, Rothmund-Thomson syndrome, and systemic sclerosis.1,2 EPS has also been described in patients receiving penicillamine for prolonged periods.2-3 In Down syndrome, EPS has been reported to be more generalized and recalcitrant to treatment than in other patients.2,4 Unfortunately, no standard therapies exist, and treatment is challenging.
- Subjects
DOWN syndrome; HEPATOLENTICULAR degeneration
- Publication
International Journal of Dermatology, 2021, Vol 60, Issue 5, p611
- ISSN
0011-9059
- Publication type
Article
- DOI
10.1111/ijd.15321