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Current Dermatology Reports
Published in: Current Dermatology Reports 1/2014

01-03-2014 | Epidemiology (A Armstrong, Section Editor)

Epidemiology of Hidradenitis Suppurativa: Prevalence, Pathogenesis, and Factors Associated with the Development of HS

Authors: Inge E. Deckers, Hessel H. van der Zee, Errol P. Prens

Published in: Current Dermatology Reports | Issue 1/2014

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Abstract

Hidradenitis suppurativa is a common skin disease with an estimated prevalence of approximately 1 %. The disease is characterized by painful nodules localized mainly in the inverse body areas, such as the axillae and groin. The pathogenesis of HS is still not fully understood, but infundibular hyperkeratosis, causing follicular occlusion, is thought to be the primary event followed by rupture with an inflammatory response. Multiple factors are associated with HS, especially smoking and obesity. More recently, the genetic background and an aberrant innate immune response have gained more attention as predisposing factors. In this review, we discuss the prevalence, pathogenesis, and relevant factors influencing the development of HS.
Literature
1.
•• Jemec GBE. Hidradenitis suppurativa. N Engl J Med. 2012;366:158–64. This article is a comprehensive review about HS and its treatments. PubMedCrossRef
2.
Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009;60:539–61.PubMedCrossRef
3.
Revuz JE, Canoui-Poitrine F, Wolkenstein P, Viallette C, Gabison G, Pouget F, et al. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol. 2008;59:596–601.PubMedCrossRef
4.
Jemec GBE, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol. 1996;35:191–4.PubMedCrossRef
5.
Canoui-Poitrine F, Revuz JE, Wolkenstein P, Viallette C, Gabison G, Pouget F, et al. Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity. J Am Acad Dermatol. 2009;61:51–7.PubMedCrossRef
6.
Revuz J. Hidradenitis suppurativa. J Eur Acad Dermatol Venereo. 2009;23:985–98.CrossRef
7.
Von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2000;14:389–92.PubMedCrossRef
8.
Mengesha YM, Holcombe TC, Hansen RC. Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol. 1999;16:292–6.PubMedCrossRef
9.
Von der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol. 2000;142:947–53.CrossRef
10.
C-W YU, Cook MG. Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands. Br J Dermatol. 1990;122:763–9.CrossRef
11.
Boer J, Weltevreden EF. Hidradenitis suppurativa or acne inversa. A clinicopathological study of early lesions. Br J Dermatol. 1996;135:721–5.PubMedCrossRef
12.
Van der Zee HH, de Ruiter L, Boer J, van den Broecke DG, den Hollander JC, Laman JD, et al. Alterations in leucocyte subsets and histomorphology in normal‐appearing perilesional skin and early and chronic hidradenitis suppurativa lesions. Br J Dermatol. 2012;166:98–106.PubMedCrossRef
13.
Danby FW, Jemec GBE, Marsch WC, von Laffert M. Preliminary findings suggest hidradenitis suppurativa may be due to defective follicular support. Br J Dermatol. 2013;168:1034–9.PubMedCrossRef
14.
von Laffert M, Helmbold P, Wohlrab J, Fiedler E, Stadie V, Marsch WC. Hidradenitis suppurativa (acne inversa): early inflammatory events at terminal follicles and at interfollicular epidermis. Exp Dermatol. 2010;19:533–7.
15.
Von Laffert M, Stadie V, Wohlrab J, Marsch WC. Hidradenitis suppurativa/acne inversa: bilocated epithelial hyperplasia with very different sequelae. Br J Dermatol. 2011;164:367–71.CrossRef
16.
Van der Zee HH, de Ruiter L, van den Broecke DG, Dik WA, Laman JD, Prens EP. Elevated levels of tumour necrosis factor (TNF)‐α, interleukin (IL)‐1β and IL‐10 in hidradenitis suppurativa skin: a rationale for targeting TNF‐α and IL‐1β. Br J Dermatol. 2011;164:1292–8.PubMedCrossRef
17.
Kimball AB, Kerdel F, Adams D, Mrowietz U, Gelfand JM, Gniadecki R, et al. Adalimumab for the treatment of moderate to severe hidradenitis SuppurativaA parallel randomized trial. Ann Intern Med. 2012;157:846–55.PubMedCrossRef
18.
Mekkes JR, Bos JD. Long‐term efficacy of a single course of infliximab in hidradenitis suppurativa. Br J Dermatol. 2008;158:370–4.PubMedCrossRef
19.
Rompel R, Petres J. Long‐term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg. 2000;26:638–43.PubMedCrossRef
20.
Wollina U, Koch A, Heinig B, Kittner T, Nowak A. Acne inversa (hidradenitis suppurativa): a review with a focus on pathogenesis and treatment. Indian Dermatol Online J. 2013;4:2.PubMedCentralPubMedCrossRef
21.
Van der Zee HH, Prens EP, Boer J. Deroofing: a tissue-saving surgical technique for the treatment of mild to moderate hidradenitis suppurativa lesions. J Am Acad Dermatol. 2010;63:475–80.PubMedCrossRef
22.
Esmann S, Jemec GBE. Psychosocial impact of hidradenitis suppurativa: a qualitative study. Acta Derm Venereol. 2011;91:328–32.PubMedCrossRef
23.
Von der Werth JM, Jemec GBE. Morbidity in patients with hidradenitis suppurativa. Br J Dermatol. 2001;144:809–13.PubMedCrossRef
24.
Onderdijk AJ, van der Zee HH, Esmann S, Lophaven S, Dufour DN, Jemec GBE, et al. Depression in patients with hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2013;27:473–8
25.
Kurek A, Peters EMJ, Chanwangpong A, Sabat R, Sterry W, Schneider-Burrus S. Profound disturbances of sexual health in patients with acne inversa. J Am Acad Dermatol. 2012;67:422–8.PubMedCrossRef
26.
Fitzsimmons JS, Guilbert PR, Fitzsimmons EM. Evidence of genetic factors in hidradenitis suppurativa. Br J Dermatol. 1985;113:1–8.PubMedCrossRef
27.
Cosmatos I, Matcho A, Weinstein R, Montgomery MO, Stang P. Analysis of patient claims data to determine the prevalence of hidradenitis suppurativa in the United States. J Am Acad Dermatol. 2013;69:819
28.
• 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. 2012;133:97–103. This was the first study that investigated the incidence of HS. PubMedCentralPubMedCrossRef
29.
• Van der Zee HH, Laman JD, Boer J, Prens EP. Hidradenitis suppurativa: viewpoint on clinical phenotyping, pathogenesis and novel treatments. Exp Dermatol. 2012;21:735–9. This comprehensive review article presents a new, well-explained hypothesis on the pathogenesis of HS. PubMed
30.
31.
Kamp S, Fiehn AM, Stenderup K, Rosada C, Pakkenberg B, Kemp K, et al. Hidradenitis suppurativa: a disease of the absent sebaceous gland? Sebaceous gland number and volume are significantly reduced in uninvolved hair follicles from patients with hidradenitis suppurativa. Br J Dermatol. 2011;164:1017–22.PubMedCrossRef
32.
Pink AE, Simpson MA, Brice GW, Smith CH, Desai N, Mortimer PS, et al. PSENEN and NCSTN mutations in familial hidradenitis suppurativa (Acne Inversa). J Invest Dermatol. 2011;131:1568–70.PubMedCrossRef
33.
•• Pink AE, Simpson MA, Desai N, Trembath RC, Barker JNW. γ-secretase mutations in hidradenitis suppurativa: new insights into disease pathogenesis. J Invest Dermatol. 2012;133:601–7. This article gives an excellent overview about the mutations found in HS. PubMedCrossRef
34.
Wang B, Yang W, Wen W, Sun J, Su B, Liu B, et al. γ-secretase gene mutations in familial acne inversa. Science. 2010;330:1065.PubMedCrossRef
35.
Melnik BC, Plewig G. Impaired Notch‐MKP‐1 signalling in hidradenitis suppurativa: an approach to pathogenesis by evidence from translational biology. Exp Dermatol. 2013;22:172–7.PubMedCrossRef
36.
Van der Zee HH, Van Der Woude CJ, Florencia EF, Prens EP. Hidradenitis suppurativa and inflammatory bowel disease: are they associated? Results of a pilot study. Br J Dermatol. 2010;162:195–7.PubMedCrossRef
37.
Nazary M, van der Zee HH, Prens EP, Folkerts G, Boer J. Pathogenesis and pharmacotherapy of Hidradenitis suppurativa. Eur J Pharmacol. 2011;672:1–8.PubMedCrossRef
38.
Schlapbach C, Hänni T, Yawalkar N, Hunger RE. Expression of the IL-23/Th17 pathway in lesions of hidradenitis suppurativa. J Am Acad Dermatol. 2011;65:790–8.PubMedCrossRef
39.
Van der Zee HH, Laman JD, de Ruiter L, Dik WA, Prens EP. Adalimumab (antitumour necrosis factor‐α) treatment of hidradenitis suppurativa ameliorates skin inflammation: an in situ and ex vivo study. Br J Dermatol. 2012;166:298–305.PubMedCrossRef
40.
Dréno B, Khammari A, Brocard A, Moyse D, Blouin E, Guillet G, et al. Hidradenitis suppurativa: the role of deficient cutaneous innate immunity. Arch Dermatol. 2012;148:182.PubMedCrossRef
41.
Hunger RE, Surovy AM, Hassan AS, Braathen LR, Yawalkar N. Toll‐like receptor 2 is highly expressed in lesions of acne inversa and colocalizes with C‐type lectin receptor. Br J Dermatol. 2008;158:691–7.PubMedCrossRef
42.
Hofmann SC, Saborowski V, Lange S, Kern WV, Bruckner-Tuderman L, Rieg S. Expression of innate defense antimicrobial peptides in hidradenitis suppurativa. J Am Acad Dermatol. 2012;66:966–74.PubMedCrossRef
43.
Schlapbach C, Yawalkar N, Hunger RE. Human β-defensin-2 and psoriasin are overexpressed in lesions of acne inversa. J Am Acad Dermatol. 2009;61:58–65.PubMedCrossRef
44.
Emelianov VU, Bechara FG, Gläser R, Langan EA, Taungjaruwinai WM, Schröder JM, et al. Immunohistological pointers to a possible role for excessive cathelicidin (LL‐37) expression by apocrine sweat glands in the pathogenesis of hidradenitis suppurativa/acne inversa. Br J Dermatol. 2012;166:1023–34.PubMedCrossRef
45.
Wolk K, Warszawska K, Hoeflich C, Witte E, Schneider-Burrus S, Witte K, et al. Deficiency of IL-22 contributes to a chronic inflammatory disease: pathogenetic mechanisms in acne inversa. J Immunol. 2011;186:1228–39.PubMedCrossRef
46.
Mortimer PS, Dawber RP, Gales MA, Moore RA. Mediation of hidradenitis suppurativa by androgens. Br Med J (Clin Res Ed). 1986;292:245.CrossRef
47.
Jemec GBE. The symptomatology of hidradenitis suppurativa in women. Br J Dermatol. 1988;119:345–50.PubMedCrossRef
48.
Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre‐and postmenopausal women with hidradenitis suppurativa. Br J Dermatol. 1996;134:1057–9.PubMedCrossRef
49.
Harrison BJ, Read GF, Hughes LE. Endocrine basis for the clinical presentation of hidradenitis suppurativa. Br J Surg. 1988;75:972–5.PubMedCrossRef
50.
Sartorius K, Emtestam L, Jemec GBE, Lapins J. Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity. Br J Dermatol. 2009;161:831–9.PubMedCrossRef
51.
König A, Lehmann C, Rompel R, Happle R. Cigarette smoking as a triggering factor of hidradenitis suppurativa. Dermatology. 1999;198:261–4.PubMedCrossRef
52.
Kurzen H, Kurokawa I, Jemec GBE, Emtestam L, Sellheyer K, Giamarellos-Bourboulis EJ, et al. What causes hidradenitis suppurativa? Exp Dermatol. 2008;17:455–6.PubMedCrossRef
53.
54.
Blandina P, Fantozzi R, Mannaioni PF, Masini E. Characteristics of histamine release evoked by acetylcholine in isolated rat mast cells. J Physiol. 1980;301:281–93.PubMed
55.
Sørensen LT, Nielsen HB, Kharazmi A, Gottrup F. Effect of smoking and abstention on oxidative burst and reactivity of neutrophils and monocytes. Surgery. 2004;136:1047–53.PubMedCrossRef
56.
Aoshiba K, Nagai A, Yasui S, Konno K. Nicotine prolongs neutrophil survival by suppressing apoptosis. J Lab Clin Med. 1996;127:186–94.PubMedCrossRef
57.
Hana A, Booken D, Henrich C, Gratchev A, Maas-Szabowski N, Goerdt S, et al. Functional significance of non-neuronal acetylcholine in skin epithelia. Life Sci. 2007;80:2214–20.PubMedCrossRef
58.
Matusiak Ł, Bieniek A, Szepietowski JC. Hidradenitis suppurativa and associated factors: still unsolved problems. J Am Acad Dermatol. 2009;61:362–5.PubMedCrossRef
59.
60.
Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007;56:901–16.PubMedCrossRef
61.
Coppack SW. Pro-inflammatory cytokines and adipose tissue. Proc Nutr Soc Lond 2001;60:349–56.
62.
Yazdanyar S, Jemec GBE. Hidradenitis suppurativa: a review of cause and treatment. Curr Opin Infect Dis. 2011;24:118–23.PubMedCrossRef
63.
Van der Zee HH, Boer J, Prens EP, Jemec GBE. The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology. 2009;219:143–7.PubMedCrossRef
64.
Bettoli V, Zauli S, Borghi A, Toni G, Minghetti S, Ricci M, et al. Oral clindamycin and rifampicin in the treatment of hidradenitis suppurativa‐acne inversa: a prospective study on 23 patients. J Eur Acad Dermatol Venereol. 2013. doi: 10.​1111/​jdv.​12127.​
65.
Morgan WP, Leicester G. The role of depilation and deodorants in hidradenitis suppurativa. Arch Dermatol. 1982;118:101.PubMedCrossRef
66.
Dufour DN, Bryld LE, Jemec GBE. Hidradenitis suppurativa complicating naevus comedonicus: the possible influence of mechanical stress on the development of hidradenitis suppurativa. Dermatology. 2010;220:323–5.PubMedCrossRef
67.
De Winter K, van der Zee HH, Prens EP. Is mechanical stress an important pathogenic factor in hidradenitis suppurativa? Exp Dermatol. 2012;21:176–7.PubMedCrossRef
68.
Chicarilli ZN. Follicular occlusion triad: hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. Ann Plast Surg. 1987;18:230–7.PubMedCrossRef
69.
Scheinfeld NS. A case of dissecting cellulitis and a review of the literature. Dermatol Online J. 2003;9:8
70.
71.
Canoui-Poitrine F, Le Thuaut A, Revuz JE, Viallette C, Gabison G, Poli F, et al. Identification of three hidradenitis suppurativa phenotypes: latent class analysis of a cross-sectional study. J Invest Dermatol. 2013;133:1506–11
72.
Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. The differential diagnosis and comorbidity of hidradenitis suppurativa and perianal Crohn’s disease. Int J Colorectal Dis. 1993;8:117–9.PubMedCrossRef
73.
Yazdanyar S, Miller IM, Jemec GB. Hidradenitis suppurativa and Crohn’s disease: two cases that support an association. Acta Dermatovenerol Alp Panonica Adriat. 2010;19:23–5
74.
Hsiao JL, Antaya RJ, Berger T, Maurer T, Shinkai K, Leslie KS. Hidradenitis suppurativa and concomitant pyoderma gangrenosum: a case series and literature review. Arch Dermatol. 2010;146:1265–70.PubMed
75.
Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatology Venereol. 2009;23:1008–17.CrossRef
76.
Bhalla R, Sequeira W. Arthritis associated with hidradenitis suppurativa. Ann Rheum Dis. 1994;53:64–6.PubMedCrossRef
77.
Leybishkis B, Fasseas P, Ryan KF, Roy R. Hidradenitis suppurativa and acne conglobata associated with spondyloarthropathy. Am J Med Sci. 2001;321:195–7.PubMedCrossRef
78.
Thein M, Hogarth MB, Acland K. Seronegative arthritis associated with the follicular occlusion triad. Clin Exp Dermatol. 2004;29:550–2.PubMedCrossRef
Metadata
Title
Epidemiology of Hidradenitis Suppurativa: Prevalence, Pathogenesis, and Factors Associated with the Development of HS
Authors
Inge E. Deckers
Hessel H. van der Zee
Errol P. Prens
Publication date
01-03-2014
Publisher
Springer US
Published in
Current Dermatology Reports / Issue 1/2014
Electronic ISSN: 2162-4933
DOI
https://doi.org/10.1007/s13671-013-0064-8

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