Hidradenitis Suppurativa and Down Syndrome: Systematic Review and Meta-Analysis
Main Article Content
Keywords
hidradenitis suppurativa, dermatology, down syndrome
Abstract
Introduction: Hidradenitis suppurativa (HS) is a chronic, inflammatory skin disease, characterized by the presence of painful lesions and recurrent abscesses, and nodules. These lesions can later rupture to form sinus tracts and fistulas. The exact etiology of HS is currently unknown, however a link between genetic, social and environmental factors has been postulated in recent studies. A number of studies have shown a significant association between HS and down syndrome (DS), however the relationship between HS and DS is unclear. We conducted a systematic review and meta-analysis to test for any association between HS and down syndrome.
Methods: A systematic review of existing studies was performed and data was pooled for meta-analysis. Reviews, abstracts and case reports were excluded. Eligible studies were those which investigated HS in cases of down syndrome. Studies that reported DS cases amongst HS patients were not included in the present study.
Results: A total of 6 studies were identified from systematic database searches after applying inclusion and exclusion criteria. Pooled meta-analysis demonstrated a significant association between HS and down syndrome. The pooled proportion of HS cases in the DS group was 10.9% (95%CI,3.8%-27.6%). The pooled proportion of HS cases in the control group was 0.4% (95% CI, 0.2%-0.8%). This difference was significantly different (P<0.001). The odds ratio is 12.02(95%CI,10.91-13.23).
Conclusion: The evidence for hidradenitis suppurativa being associated with down syndrome is very limited. The data is promising however, and further prospective studies with larger cohorts are required to reaffirm the findings in the present review.
References
2. Fimmel S, Zouboulis CC. Comorbidities of hidradenitis suppurativa (acne inversa). Dermatoendocrinol. 2010;2(1):9-16. doi:10.4161/derm.2.1.12490
3. Garg A, Kirby JS, Lavian J, Lin G, Strunk A. Sex- and Age-Adjusted Population Analysis of Prevalence Estimates for Hidradenitis Suppurativa in the United States. JAMA Dermatology. 2017;153(8):760-764. doi:10.1001/jamadermatol.2017.0201
4. Roizen NJ, Patterson D. Down’s syndrome. Lancet. 2003;361(9365):1281-1289. doi:10.1016/S0140-6736(03)12987-X
5. Kazemi M, Salehi M, Kheirollahi M. Down Syndrome: Current Status, Challenges and Future Perspectives. Int J Mol Cell Med. 2016;5(3):125-133.
6. Daneshpazhooh M, Nazemi TMJ, Bigdeloo L, Yoosefi M. Mucocutaneous findings in 100 children with Down syndrome. Pediatr Dermatol. 2007;24(3):317-320. doi:10.1111/j.1525-1470.2007.00412.x
7. Firsowicz M, Boyd M, Jacks SK. Follicular occlusion disorders in Down syndrome patients. Pediatr Dermatol. 2020;37(1):219-221. doi:10.1111/pde.14012
8. 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. doi:10.1111/bjd.15770
9. Hamadah I, Haider M, Chisti M, Binamer Y. Hidradenitis Suppurativa in Down Syndrome: A Case Series. Pediatr Dermatol. 2017;34(4):461-464. doi:10.1111/pde.13188
10. Poizeau F, Sbidian E, Mircher C, et al. Prevalence and Description of Hidradenitis Suppurativa in Down Syndrome: A Cross-sectional Study of 783 Subjects. Acta Derm Venereol. 2019;99(3):351-352. doi:10.2340/00015555-3095
11. Rork JF, McCormack L, Lal K, Wiss K, Belazarian L. Dermatologic conditions in Down syndrome: A single-center retrospective chart review. Pediatr Dermatol. Published online June 10, 2020. doi:10.1111/pde.14214
12. 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. doi:10.5826/dpc.0903a03
13. Blok J, Jonkman M, Horváth 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. doi:10.1111/bjd.12887
14. Jenkins EC, Devine-Gage EA, Robakis NK, et al. Fine mapping of an Alzheimer disease-associated gene encoding beta-amyloid protein. Biochem Biophys Res Commun. 1988;151(1):1-8. doi:10.1016/0006-291x(88)90551-7
15. Herzog V, Kirfel G, Siemes C, Schmitz A. Biological roles of APP in the epidermis. Eur J Cell Biol. 2004;83(11-12):613-624. doi:10.1078/0171-9335-00401
16. Wang B, Yang W, Wen W, et al. Gamma-secretase gene mutations in familial acne inversa. Science. 2010;330(6007):1065. doi:10.1126/science.1196284
17. Melnik BC, Plewig G. Impaired Notch-MKP-1 signalling in hidradenitis suppurativa: an approach to pathogenesis by evidence from translational biology. Exp Dermatol. 2013;22(3):172-177. doi:10.1111/exd.12098
18. Rubin SS, Rimmer JH, Chicoine B, Braddock D, McGuire DE. Overweight prevalence in persons with Down syndrome. Ment Retard. 1998;36(3):175-181. doi:10.1352/0047-6765(1998)036<0175:OPIPWD>2.0.CO;2
19. Gold DA, Reeder VJ, Mahan MG, Hamzavi IH. The prevalence of metabolic syndrome in patients with hidradenitis suppurativa. J Am Acad Dermatol. 2014;70(4):699-703. doi:10.1016/j.jaad.2013.11.014
20. Miller IM, Ellervik C, Vinding GR, et al. Association of metabolic syndrome and hidradenitis suppurativa. JAMA Dermatol. 2014;150(12):1273-1280. doi:10.1001/jamadermatol.2014.1165
21. Reddy S, Strunk A, Garg A. Comparative Overall Comorbidity Burden Among Patients With Hidradenitis Suppurativa. JAMA Dermatol. 2019;155(7):797-802. doi:10.1001/jamadermatol.2019.0164
22. 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. Published online January 23, 2021:S0190-9622(21)00213-9. doi:10.1016/j.jaad.2021.01.059