Dermatologic care of patients with differences of sex development

• Type of Material: Article
• Authors: Sarah Gold, Emory University; Christina Huang, Emory University; Rakan Radi, Emory University; Pranav Gupta, Emory University; Eric Felner, Emory University; Jeehea Haw, Emory University; Krista Childress, The University of Utah; Nancy Sokkary, Emory University; Vin Tangpricha, Emory University; Michael Goodman, Emory University; Howa Yeung, Emory University
• Language: English
• Date: 2023-09-05
• Publisher: United States Government
• Publication Version: Final Published Version
• Copyright Statement: Written work prepared by employees of the Federal Government as part of their official duties is, under the U.S. Copyright Act, a “work of the United States Government” for which copyright protection under Title 17 of the United States Code is not available. As such, copyright does not extend to the contributions of employees of the Federal Government.
• Final Published Version (URL): http://dx.doi.org/10.1097/JW9.0000000000000106
• Title of Journal or Parent Work: International Journal of Women's Dermatology
• Volume: 9
• Issue: 3
• Start Page: E106
• End Page: E106
• Grant/Funding Information: Howa Yeung is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases under award numbers L30AR076081 and K23AR075888. Michael Goodman is supported by the Eunice Kennedy Shriver National Institute of Child Health and Human Development under award number R01HD092595.
• Keywords: acne; disorders of sex development; intersex; Turner syndrome; Klinefelter syndrome; congenital adrenal hyperplasia
• Research Categories: Gender Studies; Health Sciences, General

Abstract

Differences of sex development (DSD or disorders of sex development) are uncommon congenital conditions, characterized by atypical development of chromosomal, gonadal, or anatomic sex. Objective: Dermatologic care is an important component of the multidisciplinary care needed for individuals with DSD. This article discusses the most common primary dermatologic manifestations of DSD in addition to the cutaneous manifestations of hormonal and surgical therapies in individuals with DSD. Data sources: Published articles including case series and case reports on PubMed. Study selections: Selection was conducted by examining existing literature with a team of multidisciplinary specialists. Methods: Narrative review. Limitations: This article was not conducted as a systematic review. Results: In Klinefelter syndrome, refractory leg ulcers and incontinentia pigmenti have been described. Turner syndrome is associated with lymphatic malformations, halo nevi, dermatitis, and psoriasis. Virilization can be seen in some forms of congenital adrenal hyperplasia, where acne and hirsutism are common. Conclusion: Dermatologists should consider teratogenic risk for treatments of skin conditions in DSD depending on pregnancy potential. Testosterone replacement, commonly used for Klinefelter syndrome, androgen insensitivity syndrome, 5-alpha reductase deficiency, gonadal dysgenesis, or ovotesticular DSD, may cause acne.