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  • Brindley Brooks

Down Syndrome and HS

Authors: Morgan Dykman, BA; Kristen Holland, MD; Jillian Rork, MD


Hidradenitis suppurativa (HS) is seen more commonly in people with Down syndrome. We need to get the word out and make sure HS is being diagnosed and managed in the Down syndrome community!


In terms of how common it is: a population-based study of nearly 12,000 people with Down syndrome found that people with Down syndrome had over 5 times the odds of HS compared to those without Down syndrome. There are numerous smaller studies that identify HS and folliculitis as the most common skin conditions in people with Down syndrome.


Given these research findings, the US and Canadian Hidradenitis Suppurativa Foundations recommends annual HS screening in people with Down syndrome. They did not give a specific age to start screening, but we typically recommend starting around age 7-8 years. A screening for HS would mean asking a health care provider to respectfully examine the armpits, chest, and groin area for any signs of HS.


We recommend screening for HS during the school age years because HS also appears to present at a younger age in people with Down syndrome. It typically presents during the teenage years, but there are reports of children even younger than this.


We do not know exactly why HS is more common in people with Down syndrome. This is an active area of research and understanding this will provide better treatments. People with Down syndrome may have an increased amount of amyloid precursor protein (APP) since the encoding gene is on the chromosome 21. APP is expressed in the skin and may cause clogging of the pores and cysts. There are also questions about how the immune system could lead to more skin inflammation and affect the skin microbiome.


If you have Down syndrome and think you might have HS, please tell your health care provider and ask to see a dermatologist. Dermatologists are key members of the Down syndrome care team and we want to help! There are many treatments options we can talk about.


Please help us spread the word about Down syndrome and HS!


References


Garg A, Strunk A, Midura M, Papagermanos V, Pomerantz H. Prevalence of hidradenitis suppurativa among patients with Down syndrome: a population-based cross-sectional analysis. Br J Dermatol. 2018;178(3):697-703.


Sechi A, Guglielmo A, Patrizi A, et al. Disseminate Recurrent Folliculitis and Hidradenitis Suppurativa Are Associated Conditions: Results From a Retrospective Study of 131 Patients With Down Syndrome and a Cohort of 12,351 Pediatric Controls. Dermatol Pract Concept. 2019;9(3):187-194.


Firsowicz M, Boyd M, Jacks SK. Follicular occlusion disorders in Down syndrome patients. Pediatr Dermatol. 2020;37(1):219-221.


Rork JF, McCormack L, Lal K, Wiss K, Belazarian L. Dermatologic conditions in Down syndrome: A single-center retrospective chart review. Pediatr Dermatol. 2020;37(5):811-816.


Lam M, Lai C, Almuhanna N, Alhusayen R. Hidradenitis suppurativa and Down syndrome: A systematic review and meta-analysis. Pediatr Dermatol. 2020;37(6):1044-1050.


Garg A, Malviya N, Strunk A, et al. Comorbidity screening in Hidradenitis Suppurativa:

evidence-based recommendations from the US and Canadian Hidradenitis Suppurativa

Foundations. J Am Acad Dermatol. 2021.


Blok J, Jonkman M, Horvath B. The possible association of hidradenitis suppurativa and Down syndrome: is increased amyloid precursor protein expression resulting in impaired Notch signalling the missing link? Br J Dermatol. 2014;170(6):1375-1377.


Huggard D, Doherty DG, Molloy EJ. Immune Dysregulation in Children With Down Syndrome. Front Pediatr. 2020;8:73.


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