Abstract
Background
Hidradenitis suppurativa (HS) is a debilitating cutaneous disease characterized by severe painful inflammatory nodules/abscesses. At present, data regarding the epidemiology and pathophysiology of this disease are limited.
Objective
To define the prevalence and comorbidity associations of HS.
Methods
This was a cross-sectional study of EPICTM Cosmos© examining over 180 million US patients. Prevalences were calculated by demographic and odds ratios (OR) and identified comorbidity correlations.
Results
All examined metabolism-related, psychological, and autoimmune/autoinflammatory (AI) diseases correlated with HS. The strongest associations were with pyoderma gangrenosum [OR 26.56; confidence interval (CI): 24.98–28.23], Down syndrome (OR 11.31; CI 10.93–11.70), and polycystic ovarian syndrome (OR 11.24; CI 11.09–11.38). Novel AI associations were found between HS and lupus (OR 6.60; CI 6.26–6.94) and multiple sclerosis (MS; OR 2.38; CI 2.29–2.48). Cutaneous malignancies were largely not associated in the unsegmented cohort; however, among Black patients, novel associations with melanoma (OR 2.39; CI 1.86–3.08) and basal cell carcinoma (OR 2.69; CI 2.15–3.36) were identified.
Limitations
International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy.
Conclusion
This is the first study to identify correlations between HS with melanoma and basal cell carcinoma (BCC) among Black patients as well as MS and lupus in all patients with HS.
Similar content being viewed by others
References
Constantinou CA, Fragoulis GE, Nikiphorou E. Hidradenitis suppurativa: infection, autoimmunity, or both? Ther Adv Musculoskelet Dis. 2019;11:1759720X19895488.
Goldburg SR, Strober BE, Payette MJ. Hidradenitis suppurativa: epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2020;82(5):1045–58.
Nguyen TV, Damiani G, Orenstein LAV, Hamzavi I, Jemec GB. Hidradenitis suppurativa: an update on epidemiology, phenotypes, diagnosis, pathogenesis, comorbidities and quality of life. J Eur Acad Dermatol Venereol. 2021;35(1):50–61.
Tiri H, Jokelainen J, Timonen M, Tasanen K, Huilaja L. Substantially reduced life expectancy in patients with hidradenitis suppurativa: a Finnish nationwide registry study. Br J Dermatol. 2019;180(6):1543–4.
Garg A, Malviya N, Strunk A, Wright S, Alavi A, Alhusayen R, et al. Comorbidity screening in hidradenitis suppurativa: evidence-based recommendations from the US and Canadian Hidradenitis Suppurativa Foundations. J Am Acad Dermatol. 2022;86(5):1092–101.
Melnik BC, John SM, Chen W, Plewig G. T helper 17 cell/regulatory T-cell imbalance in hidradenitis suppurativa/acne inversa: the link to hair follicle dissection, obesity, smoking and autoimmune comorbidities. Br J Dermatol. 2018;179(2):260–72.
Preda-Naumescu A, Ahmed HN, Mayo TT, Yusuf N. Hidradenitis suppurativa: pathogenesis, clinical presentation, epidemiology, and comorbid associations. Int J Dermatol. 2021;60(11):e449–58.
Seyed Jafari SM, Hunger RE, Schlapbach C. Hidradenitis suppurativa: current understanding of pathogenic mechanisms and suggestion for treatment algorithm. Front Med. 2020;7:68.
Remedios KA, Zirak B, Sandoval PM, Lowe MM, Boda D, Henley E, et al. The TNFRSF members CD27 and OX40 coordinately limit T(H)17 differentiation in regulatory T cells. Sci Immunol. 2018;3(30).
Yidana DB. Hidradenitis suppurativa—the role of interleukin-17, the aryl hydrocarbon receptor and the link to a possible fungal aetiology. Med Hypotheses. 2021;149: 110530.
Lanz TV, Brewer RC, Ho PP, Moon JS, Jude KM, Fernandez D, et al. Clonally expanded B cells in multiple sclerosis bind EBV EBNA1 and GlialCAM. Nature. 2022;603(7900):321–7.
Frew JW, Navrazhina K, Marohn M, Lu PC, Krueger JG. Contribution of fibroblasts to tunnel formation and inflammation in hidradenitis suppurativa/ acne inversa. Exp Dermatol. 2019;28(8):886–91.
Dufour DN, Emtestam L, Jemec GB. Hidradenitis suppurativa: a common and burdensome, yet under-recognised, inflammatory skin disease. Postgrad Med J. 2014;90(1062):216–21 (quiz 20).
Tarabichi Y, Frees A, Honeywell S, Huang C, Naidech AM, Moore JH, Kaelber DC. The cosmos collaborative: a vendor-facilitated electronic health record data aggregation platform. ACI Open. 2021;5(1):e36–46.
Epic. Epic Cosmos: A Universe of data that drives evidence-based research and individualized patient care USA2023. https://cosmos.epic.com/. Accessed Feb 2023
Son M, Gallagher K, Lo JY, Lindgren E, Burris HH, Dysart K, et al. Coronavirus disease 2019 (COVID-19) pandemic and pregnancy outcomes in a US population. Obstet Gynecol. 2021;138(4):542–51.
Handley SC, Gallagher K, Breden A, Lindgren E, Lo JY, Son M, et al. Birth hospital length of stay and rehospitalization during COVID-19. Pediatrics. 2022;149(1).
Handley SC, Gallagher K, Lindgren E, Lo JY, Burris HH, Dysart KC, et al. Postpartum length of stay and hospital readmission before and during the coronavirus disease 2019 (COVID-19) pandemic. Obstet Gynecol. 2022;139(3):381–90.
Plumb ID, Feldstein LR, Barkley E, Posner AB, Bregman HS, Hagen MB, Gerhart JL. Effectiveness of COVID-19 mRNA vaccination in preventing COVID-19-associated hospitalization among adults with previous SARS-CoV-2 infection—United States, June 2021-February 2022. MMWR Morb Mortal Wkly Rep. 2022;71(15):549–55.
Kranyak A, Rork J, Levy J, Burdick TE. Alopecia areata and thyroid screening in down syndrome: leveraging Epic Cosmos Dataset. J Am Acad Dermatol. 2023;89:360.
Krimpuri R, Youngs C, Emerman C. Initiation of medication for alcohol use disorder for inpatients with alcohol withdrawal syndromes. J Stud Alcohol Drugs. 2022.
Pham HH, Sandberg N, Trinkl J, Thayer J. Racial and ethnic differences in rates and age of diagnosis of autism spectrum disorder. JAMA Netw Open. 2022;5(10): e2239604.
Kim EG, Kaelber DC. Phenotypic prevalence of obesity and metabolic syndrome among an underdiagnosed and underscreened population of over 50 million children and adults. Front Genet. 2022;13: 961116.
Sachdeva M, Shah M, Alavi A. Race-specific prevalence of hidradenitis suppurativa. J Cutan Med Surg. 2021;25(2):177–87.
Shlyankevich J, Chen AJ, Kim GE, Kimball AB. Hidradenitis suppurativa is a systemic disease with substantial comorbidity burden: a chart-verified case-control analysis. J Am Acad Dermatol. 2014;71(6):1144–50.
Phan K, Huo YR, Smith SD. Hidradenitis suppurativa and psychiatric comorbidities, suicides and substance abuse: systematic review and meta-analysis. Ann Transl Med. 2020;8(13):821.
Kohorst JJ, Kimball AB, Davis MD. Systemic associations of hidradenitis suppurativa. J Am Acad Dermatol. 2015;73(5 Suppl 1):S27-35.
Calao M, Wilson JL, Spelman L, Billot L, Rubel D, Watts AD, Jemec GBE. Hidradenitis suppurativa (HS) prevalence, demographics and management pathways in Australia: a population-based cross-sectional study. PLoS ONE. 2018;13(7): e0200683.
Sabat R, Jemec GBE, Matusiak Ł, Kimball AB, Prens E, Wolk K. Hidradenitis suppurativa. Nat Rev Dis Primers. 2020;6(1):18.
Yang JH, Moon J, Kye YC, Kim KJ, Kim MN, Ro YS, et al. Demographic and clinical features of hidradenitis suppurativa in Korea. J Dermatol. 2018;45(12):1389–95.
Deckers IE, Mihajlović D, Prens EP, Boer J. Hidradenitis suppurativa: a pilot study to determine the capability of patients to self-assess their Hurley stage. Br J Dermatol. 2015;172(5):1418–9.
Ingram JR, Jenkins-Jones S, Knipe DW, Morgan CLI, Cannings-John R, Piguet V. Population-based Clinical Practice Research Datalink study using algorithm modelling to identify the true burden of hidradenitis suppurativa. Br J Dermatol. 2018;178(4):917–24.
Cices A, Ibler E, Majewski S, Huynh T, Sable KA, Brieva J, et al. Hidradenitis suppurativa association at the time of, or subsequent to, diagnosis of inflammatory bowel disease in a large U.S. patient population. J Eur Acad Dermatol Venereol. 2017;31(7):e311–e2.
Tannenbaum R, Strunk A, Garg A. Overall and subgroup prevalence of pyoderma gangrenosum among patients with hidradenitis suppurativa: a population-based analysis in the United States. J Am Acad Dermatol. 2019;80(6):1533–7.
Deckers IE, Benhadou F, Koldijk MJ, Del Marmol V, Horváth B, Boer J, et al. Inflammatory bowel disease is associated with hidradenitis suppurativa: results from a multicenter cross-sectional study. J Am Acad Dermatol. 2017;76(1):49–53.
Phan K, Charlton O, Smith SD. Hidradenitis suppurativa and metabolic syndrome—systematic review and adjusted meta-analysis. Int J Dermatol. 2019;58(10):1112–7.
Lee JH, Kwon HS, Jung HM, Kim GM, Bae JM. Prevalence and comorbidities associated with hidradenitis suppurativa in Korea: a nationwide population-based study. J Eur Acad Dermatol Venereol. 2018;32(10):1784–90.
Mintoff D, Benhadou F, Pace NP, Frew JW. Metabolic syndrome and hidradenitis suppurativa: epidemiological, molecular, and therapeutic aspects. Int J Dermatol. 2022;61(10):1175–86.
Jalenques I, Ciortianu L, Pereira B, D’Incan M, Lauron S, Rondepierre F. The prevalence and odds of anxiety and depression in children and adults with hidradenitis suppurativa: systematic review and meta-analysis. J Am Acad Dermatol. 2020;83(2):542–53.
Jung JM, Lee KH, Kim YJ, Chang SE, Lee MW, Choi JH, et al. Assessment of overall and specific cancer risks in patients with hidradenitis suppurativa. JAMA Dermatol. 2020;156(8):844–53.
Crowley JJ, Mekkes JR, Zouboulis CC, Scheinfeld N, Kimball A, Sundaram M, et al. Association of hidradenitis suppurativa disease severity with increased risk for systemic comorbidities. Br J Dermatol. 2014;171(6):1561–5.
Poizeau F, Sbidian E, Mircher C, Rebillat AS, Chosidow O, Wolkenstein P, et al. Prevalence and description of hidradenitis suppurativa in Down syndrome: a cross-sectional study of 783 subjects. Acta Derm Vener. 2019;99(3):351–2.
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 Dermatol. 2017;153(8):760–4.
Sechi A, Guglielmo A, Patrizi A, Savoia F, Cocchi G, Leuzzi M, Chessa MA. 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):187–94.
Vazquez BG, Alikhan A, Weaver AL, Wetter DA, Davis MD. Incidence of hidradenitis suppurativa and associated factors: a population-based study of Olmsted County. Minnesota J Invest Dermatol. 2013;133(1):97–103.
Shavit E, Dreiher J, Freud T, Halevy S, Vinker S, Cohen AD. Psychiatric comorbidities in 3207 patients with hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2015;29(2):371–6.
Kwak SG, Kim JH. Central limit theorem: the cornerstone of modern statistics. Korean J Anesthesiol. 2017;70(2):144–56.
Altman DG, Bland JM. How to obtain the confidence interval from a P value. Br Med J. 2011;343: d2090.
Benjamini Y, Hochberg Y. Controlling the false discovery rate: a practical and powerful approach to multiple testing. J R Stat Soc Ser B Methodol. 1995;57(1):289–300.
Tsugawa Y, Jena AB, Orav EJ, et al. Age and sex of surgeons and mortality of older surgical patients: observational study. Br Med J. 2018;361: k1343. https://doi.org/10.1136/bmj.k1343.AORNjournal.2018;108(6):695-700.
Carmona-Rivera C, O'Neil LJ, Patino-Martinez E, Shipman WD, Zhu C, Li QZ, et al. Autoantibodies Present in Hidradenitis Suppurativa Correlate with Disease Severity and Promote the Release of Proinflammatory Cytokines in Macrophages. J Invest Dermatol. 2022;142(3 Pt B):924–35.
Pironi D, Caruso F, Panarese A, Vendettuoli M, Mascagni D, Moraldi L, Filippini A. Chronic hidradenitis suppurativa in the inguinal, perineal and scrotal regions. A case report and review of the literature. Ann Ital Chir. 2010;81(6):465–70.
Saygın D, Syed AU, Lowder CY, Srivastava S, Maya JJ, Hajj-Ali RA. Characteristics of inflammatory eye disease associated with hidradenitis suppurativa. Eur J Rheumatol. 2018;5(3):165–8.
Posso-De Los Rios CJ, Sarfo A, Ghias M, Alhusayen R, Hamzavi I, Lowes MA, Alavi A. Proceeding report of the third symposium on hidradenitis suppurativa advances (SHSA) 2018. Exp Dermatol. 2019;28(7):769–75.
Carmona-Rivera C, O’Neil LJ, Patino-Martinez E, Shipman WD, Zhu C, Li QZ, et al. Autoantibodies present in hidradenitis suppurativa correlate with disease severity and promote the release of proinflammatory cytokines in macrophages. J Invest Dermatol. 2022;142(3 Pt B):924–35.
Pironi D, Caruso F, Panarese A, Vendettuoli M, Mascagni D, Moraldi L, Filippini A. Chronic hidradenitis suppurativa in the inguinal, perineal and scrotal regions A case report and review of the literature. Ann Ital Chir. 2010;81(6):465–70.
Saygın D, Syed AU, Lowder CY, Srivastava S, Maya JJ, Hajj-Ali RA. Characteristics of inflammatory eye disease associated with hidradenitis suppurativa. Euro J Rheumat. 2018;5(3):165–8.
Ross Y, Ballou S. Association of hidradenitis suppurativa with autoimmune disease and autoantibodies. Rheumatol Adv Pract. 2022;6(2):rkab108.
Lee JW, Heo YW, Lee JH, Lee S. Epidemiology and comorbidity of hidradenitis suppurativa in Korea for 17 years: a nationwide population-based cohort study. J Dermatol. 2023;50(6):778–86.
Barber MRW, Drenkard C, Falasinnu T, Hoi A, Mak A, Kow NY, et al. Global epidemiology of systemic lupus erythematosus. Nat Rev Rheumatol. 2021;17(9):515–32.
Jorge A, Castro VM, Barnado A, Gainer V, Hong C, Cai T, et al. Identifying lupus patients in electronic health records: development and validation of machine learning algorithms and application of rule-based algorithms. Semin Arthritis Rheum. 2019;49(1):84–90.
Rees F, Doherty M, Grainge MJ, Lanyon P, Zhang W. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology. 2017;56(11):1945–61.
Izmirly PM, Parton H, Wang L, McCune WJ, Lim SS, Drenkard C, et al. Prevalence of systemic lupus erythematosus in the United States: estimates from a meta-analysis of the Centers for Disease Control and Prevention National Lupus Registries. Arthritis Rheumatol. 2021;73(6):991–6.
Ingram JR. The genetics of hidradenitis suppurativa. Dermatol Clin. 2016;34(1):23–8.
Sachdeva M, Mufti A, Zaaroura H, Abduelmula A, Lansang RP, Bagit A, Alhusayen R. Squamous cell carcinoma arising within hidradenitis suppurativa: a literature review. Int J Dermatol. 2021;60(11):e459–65.
Agbai ON, Buster K, Sanchez M, Hernandez C, Kundu RV, Chiu M, et al. Skin cancer and photoprotection in people of colOR a review and recommendations for physicians and the public. J Am Acad Dermatol. 2014;70(4):748–62.
Jourabchi N, Fischer AH, Cimino-Mathews A, Waters KM, Okoye GA. Squamous cell carcinoma complicating a chronic lesion of hidradenitis suppurativa: a case report and review of the literature. Int Wound J. 2017;14(2):435–8.
Penno CA, Jäger P, Laguerre C, Hasler F, Hofmann A, Gass SK, et al. Lipidomics profiling of hidradenitis suppurativa skin lesions reveals lipoxygenase pathway dysregulation and accumulation of proinflammatory leukotriene B4. J Invest Dermatol. 2020;140(12):2421-32.e10.
Matsushita M, Pearce EJ. Disrupting metabolism to treat autoimmunity. Science. 2018;360(6387):377–8.
Yu W, Barrett J, Liu P, Parameswaran A, Chiu ES, Lu CP. Novel evidence of androgen receptor immunoreactivity in skin tunnels of hidradenitis suppurativa: assessment of sex and individual variability. Br J Dermatol. 2021;185(4):855–8.
Navrazhina K, Garcet S, Zheng X, Hur HB, Frew JW, Krueger JG. High inflammation in hidradenitis suppurativa extends to perilesional skin and can be subdivided by lipocalin-2 expression. J Allergy Clin Immunol. 2022;149(1):135-44.e12.
Tzellos T, Zouboulis CC. Which hidradenitis suppurativa comorbidities should I take into account? Exp Dermatol. 2022;31(Suppl 1):29–32.
Ingram JR. The epidemiology of hidradenitis suppurativa. Br J Dermatol. 2020;183(6):990–8.
Penninx B, Lange SMM. Metabolic syndrome in psychiatric patients: overview, mechanisms, and implications. Dialogues Clin Neurosci. 2018;20(1):63–73.
Loevinger BL, Muller D, Alonso C, Coe CL. Metabolic syndrome in women with chronic pain. Metab Clin Exp. 2007;56(1):87–93.
Kwak Y, Kim Y, Kwon SJ, Chung H. Mental health status of adults with cardiovascular or metabolic diseases by gender. Int J Environ Res Public Health. 2021;18(2):514.
Moreau M, Benhaddou S, Dard R, Tolu S, Hamzé R, Vialard F, et al. Metabolic diseases and down syndrome: how are they linked together? Biomedicines. 2021;9(2):221.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Ethics approval
Not applicable.
Funding
None.
Availability of data and material
This is publicly available data provisioned by EpicTM.
Code availability
All codes used in this study have been identified in the text.
Conflicts of interest
The authors have no conflicts of interest to declare.
Reprint requests
Hilliard T. Brydges
Author contributions
E.C., A.A., A.C., R.B., and C.P. were involved in critically reviewing the study design and proposal as well as supervising the work. H.B. performed data collection and analysis. R.B. validated the analysis approach and findings. H.B., O.O., R.F., and J.B. contributed to the literature review and data collection, and H.B., O.O., and R.F. drafted the manuscript and designed the figures. All authors contributed to the critical review of the manuscript including, text and figure review, editing, and final approval.
Consent to participate
Not applicable.
Consent for publication
Not applicable.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Brydges, H.T., Onuh, O.C., Friedman, R. et al. Autoimmune, Autoinflammatory Disease and Cutaneous Malignancy Associations with Hidradenitis Suppurativa: A Cross-Sectional Study. Am J Clin Dermatol (2024). https://doi.org/10.1007/s40257-024-00844-5
Accepted:
Published:
DOI: https://doi.org/10.1007/s40257-024-00844-5