eMedicine Specialties > Dermatology > Diseases of the Adnexa

Hidradenitis Suppurativa

Author: Marina Jovanovic, MD, PhD, Chief of Dermatology Ward and Contact Dermatitis Investigative Unit, Clinic of Dermatoveneroleogic Diseases, Clinical Center, Novi Sad, Serbia; Professor in Dermatology, Medical Faculty, University of Novi Sad, Vojvodina, Serbia
Coauthor(s): George Kihiczak, MD, Clinical Associate Professor, Department of Dermatology, New Jersey Medical School and University Hospital; Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: May 12, 2010

Introduction

Background

Hidradenitis suppurativa (HS) is a disorder of the terminal follicular epithelium in the apocrine gland–bearing skin. Hidradenitis suppurativa is characterized by comedolike follicular occlusion, chronic relapsing inflammation, mucopurulent discharge, and progressive scarring.

Pathophysiology

Hidradenitis suppurativa has traditionally been considered a disorder of the apocrine glands. Hidradenitis suppurativa was first described as a distinct entity in 1839, when Velpeau reported a patient with the superficial abscess formation in the axillary, mammary, and perianal regions.1 In 1854, Verneuil associated the suppurative process with the sweat glands, and the condition was given its current name. For many years, the condition was described as Verneuil disease, but it subsequently became known as hidradenitis suppurativa. Not having performed any histopathologic studies himself, Verneuil conceded that his conclusion was based purely on the characteristic distribution of the condition.2

In 1922, Schiefferdecker classified the sweat glands as eccrine and apocrine, and he subsequently localized hidradenitis suppurativa to the apocrine glands.3 In 1939, Brunsting provided a detailed description of the histologic features of hidradenitis suppurativa. He observed the primary cellular reaction in the lumen of the apocrine glands and in the neighboring periglandular connective tissue. Detailing the clinical features of the disease, Brunsting highlighted its frequent association with acne. He noted that hidradenitis suppurativa, dissecting cellulitis of the scalp and the neck, and acne conglobata commonly occur in the same patient. He thought that the central pathogenetic event in all 3 conditions was a tendency for follicular hyperkeratinization with secondary bacterial infection.4

In 1956, Pillsbury et al combined acne conglobata, hidradenitis suppurativa, and dissecting cellulitis under the term follicular occlusion triad.5 The only flaw in their concept was the focus on apocrine sweat gland involvement. In 1975, Plewig and Kligman added pilonidal sinus as another component to the ensemble, and they introduced the term acne tetrad.6 Plewig and Kligman pointed out that hidradenitis suppurativa is a misnomer because of the lack of apocrine gland involvement, but they did not present a detailed explanation. In 1989, Plewig and Steger suggested the term acne inversa as an inclusive and accurate name for what was previously called the follicular occlusion triad, or follicular occlusion tetrad.7 Eventually, hidradenitis suppurativa was accepted as an acneiform disorder that begins with follicular occlusion rather than an infection of the sweat glands.8,9,10

Hidradenitis suppurativa is actually a defect of the follicular epithelium; therefore, all outdated synonyms for this disease, including hidradenitis suppurativa, which we now call acne inversa, should be excluded from current usage. The term acne inversa links the pathogenesis to acne and reflects the fact that it is an expression of follicular occlusion in localizations inverse to acne vulgaris.11

Frequency

United States

In the United States, the prevalence of hidradenitis suppurativa appears to be 1-2% in the general population.

International

The prevalence of hidradenitis suppurativa appears to be 1% of the general population10,12 ; it was 4% in a group of young adults who were treated at a clinic for sexually transmitted diseases.12 A 2008 study showed that the prevalence among persons aged 55 years and older was significantly lower than in younger age groups (0.5% vs 1.4%).13

Hidradenitis suppurativa is probably more common than once thought, but the diagnosis is frequently ignored or missed. Hidradenitis suppurativa has a worldwide distribution, although hot, humid environments tend to support its development.

Mortality/Morbidity

The death rate is similar to that in the general adult population.

Although rare complications of the disease are described, little is known about the typical effects of hidradenitis suppurativa. Hidradenitis suppurativa is a chronic disabling disorder that relentlessly progresses and leads to keloids, contractures, and immobility; patients can become outcasts or at least have difficulty in making social contact.

  • In the assessment of socioeconomic effects, Jemec et al noted that the most common problem in both sexes was soreness and that the average patient lost 2.7 days of work per year.14 In comparison, in Denmark, the average number of workdays lost for all reasons was 7.5 days per year per employed person, whereas patients with eczema used 4 weeks of sick leave per year because of their disease. Although hidradenitis suppurativa entails less morbidity than perhaps hand eczema, with regard to its prevalence, it significantly contributes to the average number of workdays lost (Danish National Statistical Office, Copenhagen, Denmark, Jemec GB, written communication, 1996).14
  • Jemec et al also found sex differences in morbidity. Women lost significantly more days of work (mean, 2.9 d) than men (mean, 1.7 d); this finding suggests that women have either a more fulminant course or that other contributory factors remain unrecognized. On the other hand, male patients experienced less delay in seeking care from a specialist or hospital; this finding could also suggest a more fulminant course or the presence of confounding factors.14
  • The general self-reported level of health, which is well correlated with more objective parameters of morbidity, is significantly worse among hidradenitis suppurativa patients than healthy control subjects.14
  • The mean Dermatology Life Quality Index (DLQI) score for hidradenitis suppurativa is higher than for previously studied skin diseases and is correlated with disease intensity as expressed by lesions per month. This suggests that the DLQI may be a relevant outcome measure in future therapeutic trials in hidradenitis suppurativa.15

Race

Although many authors report no racial predilection, an increased incidence is observed in blacks, possibly because blacks have a greater density of apocrine glands compared with whites.16

Sex

Although hidradenitis suppurativa is widely considered to occur more frequently in females than in males, with a ratio as high as 2-5:1,14,15 reports on sex prevalence are controversial.15,17,18

Active genitofemoral lesions occur significantly more often in females, whereas perianal involvement tends to be more common in males. No sex difference is seen in the axillary lesions. Comedones have been suggested as precursor lesions for hidradenitis suppurativa, and they appear to be equally distributed in both sexes and sites.19

Age

The onset of hidradenitis suppurativa peaks in individuals aged 11-50 years,20 with an average patient age of 23 years.15 In less than 2% of patients, the disease appears before age 11 years.21 In extremely rare cases, hidradenitis suppurativa occurs before puberty22,23 or after menopause.24

Clinical

History

Hidradenitis suppurativa usually occurs in otherwise healthy individuals, and, rarely, it can begin before puberty. The disease onset is insidious, with early symptoms of pruritus, erythema, and local hyperhidrosis. Later, the lesions become painful. Arthropathy associated with hidradenitis suppurativa may present with variable clinical features, ranging from asymmetric pauciarticular arthritis to a symmetric polyarthritis and/or polyarthralgia syndrome.25,26

Physical

The diagnosis is primarily clinical, and biopsy is rarely required, especially in well-developed lesions.20 The consensus approach has deemed that 3 key elements are required to diagnose hidradenitis suppurativa: typical lesions, characteristic distribution, and recurrence. Based on 3 premises, a set of typical lesions, called primary lesions, was compiled, as follows:

  • Painful and/or tender erythematous papules smaller than 1 cm in diameter
  • Painful and/or tender erythematous nodules larger than 1 cm in diameter
  • Painful or tender abscesses and inflamed discharging papules or nodules
  • Dermal contractures and ropelike elevation of the skin
  • Double-ended comedones

The characteristic sites were chosen in accordance with the 2 areas most frequently affected by hidradenitis suppurativa: the axillae and the groin. These areas are defined by anatomical borders and are called designated sites. Hidradenitis suppurativa is diagnosed if the patient has 1 of the following:

  • Active disease with 1 or more primary lesion in a designated site, plus a history of 3 or more discharging or painful lumps (not specified) in designated sites since age 10 years
  • Inactive disease with a history of 5 or more discharging or painful lumps (unspecified) in designated sites since age 10 years, in the absence of current primary lesions15,27

Others have based the diagnosis on a series of questions, as follows28 :

  • Is there more than a single inflamed lesion?
  • Is the course chronic, with new and recurrent lesions?
  • Are the lesions bilateral?
  • Are the lesions located primarily in the milk line?

Hidradenitis suppurativa has a predilection for the intertriginous regions. The axillary and inguinoperineal regions are most commonly affected (see the images below).Other zones that harbor terminal hair follicles and apocrine sweat glands are occasionally affected. These zones include the areola of the breast, the submammary fold, the periumbilical region, the scalp, the zygomatic and malar areas of the face, the nape of the neck, the external auditory meatus, and the shoulders.

Vulvar hidradenitis suppurativa.

Vulvar hidradenitis suppurativa.

[ CLOSE WINDOW ]
Vulvar hidradenitis suppurativa.

Vulvar hidradenitis suppurativa.


Vulvar and inguinal indurations.

Vulvar and inguinal indurations.

[ CLOSE WINDOW ]
Vulvar and inguinal indurations.

Vulvar and inguinal indurations.


The extent and the severity of the disorder vary widely. Some patients have relatively mild forms of the disease that involve only one region.16 In many patients, more than one major site is involved. One or both axillae can be affected. In addition, the inguinal region can be involved, often with spread to the scrotum, the labia, the mons pubis, the mammary or perianal region, and the buttocks.

A firm pea-size nodule appears and may spontaneously rupture, yielding a purulent discharge. The lesion then heals with fibrosis and eventual recurrence in an adjacent area. The progression from noninflamed nodules to painful, round, deep-seated lesions and subsequent scarring is highly diagnostic. The nodules tend to coalesce, and they may become infected, resulting in acute abscesses. These abscesses may temporarily resolve, or, alternatively, they may progress to multiple abscesses with persistent pain, fistula formation, and scarring. Infected ruptured apocrine glands coalesce, creating subcutaneous abscesses with discharge through multiple openings.

The inflamed nodules progress when spontaneously draining dermal sinus tracts appear. Draining sinuses represent a variant of persistent nodular hidradenitis suppurativa characterized by periodic discharge of pus or blood. If untreated, the draining sinuses persist for a long time, even years. They may seem to intermittently resolve, only to start draining again (see the images below). A draining sinus can be easily identified because of its linear or angular shape, its history of being present for a prolonged period, and its variable response to systemic isotretinoin.29 Over time, multiple abscesses and sinus tracts form a subcutaneous honeycomb. Occasionally, the involvement extends into the underlying fascia and muscles. Fibrosis, hypertrophic scarring, and induration ultimately develop.

Sinus tract.

Sinus tract.

[ CLOSE WINDOW ]
Sinus tract.

Sinus tract.


Draining sinus tract.

Draining sinus tract.

[ CLOSE WINDOW ]
Draining sinus tract.

Draining sinus tract.


Multiple open comedones and so-called bridged comedones are the hallmark finding of hidradenitis suppurativa; they frequently progress to multiple abscesses and sinus tract formations. When 2 distant cutaneous orifices are interconnected through a subcutaneous fistula, they form bridging lesions. Adenopathy is rarely associated. With advanced disease, the destruction of most glands causes the apocrine glands to decrease in number or disappear. In the axillary region, 5- to 30-cm–long confluent infiltrations develop. These infiltrating lesions are firm and tend to merge at many points. As the disease becomes chronic, scars and contractures as large as a finger develop with persistent erythema. The patient’s mobility is restricted, and the patient may not be able to fully raise his or her upper arm above the horizontal plane.

Inguinal-anogenital infiltration involves brown-red lesions with pus, blood, and a foul-smelling secretion that emerges from the numerous fistular openings. In the upper anal fold, terminal hairs emerge from thickened scars. Perianal hidradenitis suppurativa may cause pain, swelling, purulent discharge, bleeding, and fistulas. Progressive destruction of the normal skin architecture occurs; the malodorous discharge may be thin and serous or frankly purulent.

The signs of perianal hidradenitis suppurativa may be clinically identical to the cutaneous manifestations of Crohn disease. Crohn disease may be complicated by a variety of skin manifestations, and hidradenitis suppurativa has been reported to precede or complicate Crohn disease.16,30,31 In examining patients with perianal hidradenitis suppurativa, Church et al noted that Crohn disease coexisted with perianal hidradenitis suppurativa in 39% of patients.32 Local swelling and inflammation associated with Crohn disease may precipitate hidradenitis suppurativa in patients already prone to it. However, the coexistence of Crohn disease and hidradenitis suppurativa does not explain the frequent presence of axillary, groin, and buttock involvement, which may imply a constitutional or genetic predisposition to hidradenitis suppurativa in patients with rectal Crohn disease.16

The coexistence of the 2 conditions may have implications in the treatment of perianal sepsis in such patients. Each condition can mask the other. Hidradenitis suppurativa may adversely affect the clinical course of patients with Crohn disease. Furthermore, patients with both conditions are more prone to persistent sepsis and frequently require proctocolectomy and fecal diversion procedures.16  

In patients with the chronic lesions due to so-called granulomatous hidradenitis suppurativa, as well as in 60% of patients with Crohn disease, the presence of noncaseating epithelioid-type granulomas suggests that hidradenitis suppurativa and Crohn disease are chronic inflammatory conditions. Epithelioid granulomas occur in patients with a constitutive tendency to form granulomas as a part of an immunologic abnormality.30

Whether the association between hidradenitis suppurativa and Crohn disease is more than a coincidence is unclear; however, the small proportion of patients with hidradenitis suppurativa and epithelioid granulomatous inflammation may represent a group that is susceptible to systemic granulomatous disease, including Crohn disease.30,31

The association of hidradenitis suppurativa with several disorders in which poral occlusion is prominent supports the theory of a follicular origin of hidradenitis suppurativa.33 Such disorders include Fox-Fordyce disease, acanthosis nigricans,22  pityriasis rubra pilaris (PRP),34  steatocystoma multiplex, and Dowling-Degos disease.35

HIV-associated pityriasis rubra pilaris, or pityriasis rubra pilaris type VI, is a new entity reported in patients with HIV infection. HIV-associated pityriasis rubra pilaris is characterized by the cutaneous lesions of pityriasis rubra pilaris and a variable association with the lesions of acne conglobata, hidradenitis suppurativa, and lichen spinulosus.34 This disease can be designated by the broader term, HIV-associated follicular syndrome. Although the pathogenesis of pityriasis rubra pilaris type VI is unknown, follicular inflammation secondary to infection of the hair follicle bulge area by HIV has been suggested.25

Arthritis is a well-recognized, albeit uncommon, comorbidity of several chronic cutaneous inflammatory conditions that involve severe acne. Included in this group of conditions is the arthropathy associated with hidradenitis suppurativa, acne conglobata, perifolliculitis abscedens, and suffodiens capitis.25,26 Arthritis associated with acne fulminans, seronegative spondyloarthropathies of psoriatic arthritis, and reactive arthritis are well-defined clinical entities. However, arthritis associated with hidradenitis suppurativa; acne conglobata; perifolliculitis abscedens; suffodiens capitis; and synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis (SAPHO) syndrome are less well defined; these conditions may be part of the spectrum of human leukocyte antigen B27(HLA-B27)–negative spondyloarthropathies.36

The pathogenesis of the arthritis remains unknown, but it may include an inappropriate response to bacterial antigens involved in acne, or it may be some other autoimmune response.37 Arthropathy associated with hidradenitis suppurativa may manifest variable clinical features, ranging from an asymmetric pauciarticular arthritis to a symmetric polyarthritis and/or polyarthralgia syndrome. Arthropathy typically involves the large joints in the upper or lower extremities, particularly the knee joints. The axial skeleton may also be involved. In many instances, the arthropathy worsens during flares of hidradenitis suppurativa, and, conversely, it improves after hidradenitis suppurativa resolves.36

The concept of SAPHO syndrome includes 4 subgroups of osteoarticular disorders: (1) rheumatologic manifestations associated with acne conglobata or acne fulminans or inversa hidradenitis suppurativa, (2) pustulosis palmoplantaris, (3) sternocostoclavicular hyperostosis, and (4) chronic multifocal recurrent osteomyelitis. SAPHO syndrome may encompass cases described as arthropathy associated with hidradenitis suppurativa and acne conglobata. However, aseptic osteitis with hyperostosis, particularly of the thoracic joints, is a hallmark of SAPHO syndrome, and it may represent a feature that distinguishes SAPHO syndrome from the arthropathy of hidradenitis suppurativa and acne conglobata.26,38

Some reports raised the question of whether mammillary fistula or Zuska disease might be a manifestation of acne inversa.39

Pyoderma gangrenosum has been rarely associated with hidradenitis suppurativa, and this developed only after hidradenitis suppurativa had been present for at least 2 decades, just as in the authors; patient (see Media Files 1-15). Only 15 cases of coexisting pyoderma gangrenosum and hidradenitis suppurativa were found in the English-language literature. More studies are required to support impaired neutrophil function as a common etiological pathway.40

Causes

1

More on Hidradenitis Suppurativa

Overview: Hidradenitis Suppurativa
Differential Diagnoses & Workup: Hidradenitis Suppurativa
Treatment & Medication: Hidradenitis Suppurativa
Follow-up: Hidradenitis Suppurativa
Multimedia: Hidradenitis Suppurativa
References
[ CLOSE WINDOW ]

References

  1. Velpeau A. Dictionnaire de Medicine, un Repertoire des Sciences Medicales sons le Rapport, Theorique et Pratique. 2nd ed Paris. 1839:91.

  2. Verneuil A. Etudes sur les tumeurs de la peau et quelques maladies des glandes sudoripores. Arch Gen Med. 1854;4:693-705.

  3. Schiefferdecker B. Die Hautdrusen des Menschen und der Saugetierre ihre Histologishe und rassenanatomische Bedeutung Sowie die Muscularis Sexualis. Stuttgart. 1922.

  4. Brunsting HA. Hidradenitis suppurativa: abscess of the apocrine sweat glands. Arch fur Dermatol und Syph (Berlin). 1939;39:108-20.

  5. Pillsbury DM, Shelley WB, Kligman AM. Bacterial infections of the skin. In: Dermatology. Philadelphia: WB Saunders Co; 1956:482-9.

  6. Plewig G, Kligman AM. Acne: Morphogenesis and Treatment. Berlin: Springer-Verlag; 1975.

  7. Plewig G, Steger M. Acne inversa (alias acne triad, acne tetrad, or hydradenitis suppurativa). In: Marks R, Plewig G, eds. Acne and Related Disorders. London: Martin Dunitz Ltd; 1989:343-57.

  8. Yu CC, Cook MG. Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands. Br J Dermatol. Jun 1990;122(6):763-9. [Medline].

  9. Attanoos RL, Appleton MA, Douglas-Jones AG. The pathogenesis of hidradenitis suppurativa: a closer look at apocrine and apoeccrine glands. Br J Dermatol. Aug 1995;133(2):254-8. [Medline].

  10. Jemec GB, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol. Aug 1996;35(2 Pt 1):191-4. [Medline].

  11. Sellheyer K, Krahl D. "Hidradenitis suppurativa" is acne inversa! An appeal to (finally) abandon a misnomer. Int J Dermatol. Jul 2005;44(7):535-40. [Medline].

  12. Jemec GB. What's new in hidradenitis suppurativa?. J Eur Acad Dermatol Venereol. Sep 2000;14(5):340-1. [Medline].

  13. Revuz JE, Canoui-Poitrine F, Wolkenstein P, et al. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol. Oct 2008;59(4):596-601. [Medline].

  14. Jemec GB, Heidenheim M, Nielsen NH. Hidradenitis suppurativa--characteristics and consequences. Clin Exp Dermatol. Nov 1996;21(6):419-23. [Medline].

  15. von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. Sep 2000;14(5):389-92. [Medline].

  16. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl. Mar 1997;79(2):83-9. [Medline].

  17. Manolitsas T, Biankin S, Jaworski R, Wain G. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa. Gynecol Oncol. Nov 1999;75(2):285-8. [Medline].

  18. Fearfield LA, Staughton RC. Severe vulval apocrine acne successfully treated with prednisolone and isotretinoin. Clin Exp Dermatol. May 1999;24(3):189-92. [Medline].

  19. Jemec GB. Hidradenitis suppurativa. J Cutan Med Surg. Jan-Feb 2003;7(1):47-56. [Medline].

  20. Brown TJ, Rosen T, Orengo IF. Hidradenitis suppurativa. South Med J. Dec 1998;91(12):1107-14. [Medline].

  21. Palmer RA, Keefe M. Early-onset hidradenitis suppurativa. Clin Exp Dermatol. Sep 2001;26(6):501-3. [Medline].

  22. Mengesha YM, Holcombe TC, Hansen RC. Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol. Jul-Aug 1999;16(4):292-6. [Medline].

  23. Weber-LaShore A, Huppert JS. Hidradenitis suppurativa in a pre-pubertal female. J Pediatr Adolesc Gynecol. 2009;22:e53.

  24. Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre- and postmenopausal women with hidradenitis suppurativa. Br J Dermatol. Jun 1996;134(6):1057-9. [Medline].

  25. Misery I, Faure M, Claidy A. Pityriasis rubra pilaris and human immunodeficiency virus infection--type 6 pityriasis rubra pilaris?. Br J Dermatol. Dec 1996;135(6):1008-9. [Medline].

  26. Leybishkis B, Fasseas P, Ryan KF, Roy R. Hidradenitis suppurativa and acne conglobata associated with spondyloarthropathy. Am J Med Sci. Mar 2001;321(3):195-7. [Medline].

  27. von der Werth JM, Jemec GB. Morbidity in patients with hidradenitis suppurativa. Br J Dermatol. Apr 2001;144(4):809-13. [Medline].

  28. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. Apr 2009;60(4):539-61; quiz 562-3. [Medline].

  29. Jansen T, Romiti R, Plewig G, Altmeyer P. Disfiguring draining sinus tracts in a female acne patient. Pediatr Dermatol. Mar-Apr 2000;17(2):123-5. [Medline].

  30. Roy MK, Appleton MA, Delicata RJ, Sharma AK, Williams GT, Carey PD. Probable association between hidradenitis suppurativa and Crohn's disease: significance of epithelioid granuloma. Br J Surg. Mar 1997;84(3):375-6. [Medline].

  31. Martínez F, Nos P, Benlloch S, Ponce J. Hidradenitis suppurativa and Crohn's disease: response to treatment with infliximab. Inflamm Bowel Dis. Nov 2001;7(4):323-6. [Medline].

  32. 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. Sep 1993;8(3):117-9. [Medline].

  33. Jansen T, Plewig G. What's new in acne inversa (alias hidradenitis suppurativa)?. J Eur Acad Dermatol Venereol. Sep 2000;14(5):342-3. [Medline].

  34. Gonzalez-Lopez A, Velasco E, Pozo T, Del Villar A. HIV-associated pityriasis rubra pilaris responsive to triple antiretroviral therapy. Br J Dermatol. May 1999;140(5):931-4. [Medline].

  35. Kleeman D, Trueb RM, Schmid-Grendelmeier P. [Reticular pigmented anomaly of the flexures. Dowling-Degos disease of the intertrigo type in association with acne inversa]. Hautarzt. Jul 2001;52(7):642-5. [Medline].

  36. Libow LF, Friar DA. Arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens: treatment with isotretinoin. Cutis. Aug 1999;64(2):87-90. [Medline].

  37. Thein M, Hogarth MB, Acland K. Seronegative arthritis associated with the follicular occlusion triad. Clin Exp Dermatol. 2004;29:545-62.

  38. Bhosale P, Barron B, Lamki L. The "SAPHO" syndrome: a case report of a patient with unusual bone scan findings. Clin Nucl Med. Jul 2001;26(7):619-21. [Medline].

  39. Cosman BC, Al-Refaie WB. Mammillary fistula as a manifestation of acne inversa (hidradenitis suppurativa): report of two cases. J Am Coll Surg. Jun 2002;194(6):829-33. [Medline].

  40. Ah-Weng A, Langtry JA, Velangi S, Evans CD, Douglas WS. Pyoderma gangrenosum associated with hidradenitis suppurativa. Clin Exp Dermatol. Nov 2005;30(6):669-71. [Medline].

  41. Jansen T, Plewig G. Acne inversa. Int J Dermatol. Feb 1998;37(2):96-100. [Medline].

  42. Lapins J, Jarstrand C, Emtestam L. Coagulase-negative staphylococci are the most common bacteria found in cultures from the deep portions of hidradenitis suppurativa lesions, as obtained by carbon dioxide laser surgery. Br J Dermatol. Jan 1999;140(1):90-5. [Medline].

  43. Edlich RF, Winters KL, Britt LD, Long WB 3rd, Gubler KD, Drake DB. Difficult wounds: an update. J Long Term Eff Med Implants. 2005;15(3):289-302. [Medline].

  44. Kurzen H, Jung EG, Hartschuh W, Moll I, Franke WW, Moll R. Forms of epithelial differentiation of draining sinus in acne inversa (hidradenitis suppurativa). Br J Dermatol. Aug 1999;141(2):231-9. [Medline].

  45. Kurzen H, Kurokawa I, Jemec GB, et al. What causes hidradenitis suppurativa?. Exp Dermatol. May 2008;17(5):455-6; discussion 457-72. [Medline].

  46. Kurokawa I, Nishijima S, Kusumoto K, Senzaki H, Shikata N, Tsubura A. Immunohistochemical study of cytokeratins in hidradenitis suppurativa (acne inversa). J Int Med Res. Mar-Apr 2002;30(2):131-6. [Medline].

  47. Kurokawa I, Nishijima S, Suzuki K, et al. Cytokeratin expression in pilonidal sinus. Br J Dermatol. Mar 2002;146(3):409-13. [Medline].

  48. Kurokawa I, Nishimura K, Yamanaka K, Mizutani H, Tsubura A, Revuz J. Cytokeratin expression in squamous cell carcinoma arising from hidradenitis suppurativa (acne inversa). J Cutan Pathol. Sep 2007;34(9):675-8. [Medline].

  49. Jemec GB, Gniadecka M. Ultrasound examination of hair follicles in hidradenitis suppurativa. Arch Dermatol. Aug 1997;133(8):967-70. [Medline].

  50. 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. Apr 2008;158(4):691-7. [Medline].

  51. Jansen I, Altmeyer P, Piewig G. Acne inversa (alias hidradenitis suppurativa). J Eur Acad Dermatol Venereol. Nov 2001;15(6):532-40. [Medline].

  52. Wiseman MC. Hidradenitis suppurativa: a review. Dermatol Ther. 2004;17(1):50-4. [Medline].

  53. Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. Jul 2003;56(5):451-61. [Medline].

  54. Gniadecki R, Jemec GB. Lipid raft-enriched stem cell-like keratinocytes in the epidermis, hair follicles and sinus tracts in hidradenitis suppurativa. Exp Dermatol. Jun 2004;13(6):361-3. [Medline].

  55. Matusiak L, Bieniek A, Szepietowski JC. Solubile interleukin-2 receptor serum level is a useful marker of hidradenitis suppurativa clinical staging. Biomarkers. 2009;14:432-7.

  56. Sartorius K, Lapins J, Emtestam L, Jemec GB. Suggestions form uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. Br J Dermatol. 2003;149:211-3.

  57. Frohlich D, Baaske D, Glatzel M. [Radiotherapy of hidradenitis suppurativa--still valid today?]. Strahlenther Onkol. Jun 2000;176(6):286-9. [Medline].

  58. Iwasaki J, Marra DE, Fincher EF, Moy RL. Treatment of hidradenitis suppurativa with a nonablative radiofrequency device. Dermatol Surg. Jan 2008;34(1):114-7. [Medline].

  59. Bordier-Lamy F, Palot JP, Vitry F, Bernard P, Grange F. [Surgical treatment of hidradenitis suppurativa: a retrospective study of 93 cases]. Ann Dermatol Venereol. May 2008;135(5):373-9. [Medline].

  60. Buimer MG, Wobbes T, Klinkenbijl JH. Hidradenitis suppurativa. Br J Surg. Apr 2009;96(4):350-60. [Medline].

  61. Rompel R, Petres J. Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg. Jul 2000;26(7):638-43. [Medline].

  62. Bohn J, Svensson H. Surgical treatment of hidradenitis suppurativa. Scand J Plast Reconstr Surg Hand Surg. Sep 2001;35(3):305-9. [Medline].

  63. Aksakal AB, Adisen E. Hidradenitis suppurativa: importance of early treatment; efficient treatment with electrosurgery. Dermatol Surg. Feb 2008;34(2):228-31. [Medline].

  64. Boer J, van Gemert MJ. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. Jan 1999;40(1):73-6. [Medline].

  65. Tanaka A, Hatoko M, Tada H, Kuwahara M, Mashiba K, Yurugi S. Experience with surgical treatment of hidradenitis suppurativa. Ann Plast Surg. Dec 2001;47(6):636-42. [Medline].

  66. Golcman R, Golcman B, Tamura BM, Nogueira MA, Zoo CM, Germano JA. Subcutaneous fistulectomy in bridging hidradenitis suppurativa. Dermatol Surg. Oct 1999;25(10):795-8. [Medline].

  67. Bodzin JH. Laser ablation of complex perianal fistulas preserves continence and is a rectum-sparing alternative in Crohn's disease patients. Am Surg. Jul 1998;64(7):627-31; discussion 632. [Medline].

  68. Lapins J, Sartorius K, Emtestam L. Scanner-assisted carbon dioxide laser surgery: a retrospective follow-up study of patients with hidradenitis suppurativa. J Am Acad Dermatol. Aug 2002;47(2):280-5. [Medline].

  69. Madan V, Hindle E, Hussain W, August PJ. Outcomes of treatment of nine cases of recalcitrant severe hidradenitis suppurativa with carbon dioxide laser. Br J Dermatol. Dec 2008;159(6):1309-14. [Medline].

  70. Hynes PJ, Earley MJ, Lawlor D. Split-thickness skin grafts and negative-pressure dressings in the treatment of axillary hidradenitis suppurativa. Br J Plast Surg. Sep 2002;55(6):507-9. [Medline].

  71. Rehman N, Kannan RY, Hassan S, Hart NB. Thoracodorsal artery perforator (TAP) type I V-Y advancement flap in axillary hidradenitis suppurativa. Br J Plast Surg. Jun 2005;58(4):441-4. [Medline].

  72. Rhode JM, Burke WM, Cederna PS, Haefner HK. Outcomes of surgical management of stage III vulvar hidradenitis suppurativa. J Reprod Med. Jun 2008;53(6):420-8. [Medline].

  73. Bong JL, Shalders K, Saihan E. Treatment of persistent painful nodules of hidradenitis suppurativa with cryotherapy. Clin Exp Dermatol. May 2003;28(3):241-4. [Medline].

  74. Buimer MG, Ankersmit MF, Wobbes T, Klinkenbijl JH. Surgical treatment of hidradenitis suppurativa with gentamicin sulfate: a prospective randomized study. Dermatol Surg. Feb 2008;34(2):224-7. [Medline].

  75. Jamshidi M, Obermeyer RJ, Govindaraj S, Garcia A, Ghani A. Acute pancreatitis secondary to isotretinoin-induced hyperlipidemia. J Okla State Med Assoc. Feb 2002;95(2):79-80. [Medline].

  76. Hofer T, Itin PH. [Acne inversa: a dapsone-sensitive dermatosis]. Hautarzt. Oct 2001;52(10 Pt 2):989-92. [Medline].

  77. Farrell AM, Randall VA, Vafaee T, Dawber RP. Finasteride as a therapy for hidradenitis suppurativa. Br J Dermatol. Dec 1999;141(6):1138-9. [Medline].

  78. Katsanos KH, Christodoulou DK, Tsianos EV. Axillary hidradenitis suppurativa successfully treated with infliximab in a Crohn's disease patient. Am J Gastroenterol. Aug 2002;97(8):2155-6. [Medline].

  79. Lebwohl B, Sapadin AN. Infliximab for the treatment of hidradenitis suppurativa. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S275-6. [Medline].

  80. Rosi YL, Lowe L, Kang S. Treatment of hidradenitis suppurativa with infliximab in a patient with Crohn's disease. J Dermatolog Treat. Feb 2005;16(1):58-61. [Medline].

  81. Trent JT, Kerdel FA. Tumor necrosis factor alpha inhibitors for the treatment of dermatologic diseases. Dermatol Nurs. Apr 2005;17(2):97-107. [Medline].

  82. Sullivan TP, Welsh E, Kerdel FA, Burdick AE, Kirsner RS. Infliximab for hidradenitis suppurativa. Br J Dermatol. Nov 2003;149(5):1046-9. [Medline].

  83. Gupta AK, Skinner AR. A review of the use of infliximab to manage cutaneous dermatoses. J Cutan Med Surg. Mar-Apr 2004;8(2):77-89. [Medline].

  84. Grant A, Gonzalez T, Montgomery MO, Cardenas V, Kerdfel FA. Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: A randomized, double blind, placebo-control crossover trial. J Am Acad Dermatol. 2010;62:205-17.

  85. Mekkes JR, Bos JD. Long-term efficacy of a single course of infliximab in hidradenitis suppurativa. Br J Dermatol. Feb 2008;158(2):370-4. [Medline].

  86. Fardet L, Dupuy A, Kerob D, et al. Infliximab for severe hidradenitis suppurativa: transient clinical efficacy in 7 consecutive patients. J Am Acad Dermatol. Apr 2007;56(4):624-8. [Medline].

  87. Usmani N, Clayton TH, Everett S, Goodfield MD. Variable response of hidradenitis suppurativa to infliximab in four patients. Clin Exp Dermatol. Mar 2007;32(2):204-5. [Medline].

  88. Lee RA, Dommasch E, Treat J, et al. A prospective clinical trial of open-label etanercept for the treatment of hidradenitis suppurativa. J Am Acad Dermatol. Apr 2009;60(4):565-73. [Medline].

  89. Giamarellos-Bourboulis EJ, Pelekanou E, Antonopoulou A, et al. An open-label phase II study of the safety and efficacy of etanercept for the therapy of hidradenitis suppurativa. Br J Dermatol. Mar 2008;158(3):567-72. [Medline].

  90. Zangrilli A, Esposito M, Mio G, Mazzotta A, Chimenti S. Long-term efficacy of etanercept in hidradenitis suppurativa. J Eur Acad Dermatol Venereol. Nov 2008;22(10):1260-2. [Medline].

  91. Yamauchi PS, Mau N. Hidradenitis suppurativa managed with adalimumab. J Drugs Dermatol. Feb 2009;8(2):181-3. [Medline].

  92. Jemec GB. Methotrexate is of limited value in the treatment of hidradenitis suppurativa. Clin Exp Dermatol. Sep 2002;27(6):528-9. [Medline].

  93. Boer J, Jemec GB. Resorcinol peels as a possible self-treatment of painful nodules in hidradenitis suppurativa. Clin Exp Dermatol. 2010;35(1):36-40.

  94. Russ E, Castillo M. Lumbosacral epidural abscess due to hidradenitis suppurativa. AJR Am J Roentgenol. 2002;178(3):770-1.

  95. Gupta S, Kumar B. Dorsal perforation of prepuce: a common end point of severe ulcerative genital diseases?. Sex Transm Infect. Jun 2000;76(3):210-2. [Medline].

  96. Lapins J, Ye W, Nyren O, Emtestam L. Incidence of cancer among patients with hidradenitis suppurativa. Arch Dermatol. Jun 2001;137(6):730-4. [Medline].

  97. Malaguarnera M, Pontillo T, Pistone G, Succi L. Squamous-cell cancer in Verneuil's disease (hidradenitis suppurativa). Lancet. Nov 23 1996;348(9039):1449. [Medline].

  98. Maclean GM, Coleman DJ. Three fatal cases of squamous cell carcinoma arising in chronic perineal hidradenitis suppurativa. Ann R Coll Surg Engl. Oct 2007;89(7):709-12. [Medline].

  99. Crain VA, Gulati S, Bhat S, Milner SM. Marjolin's ulcer in chronic hidradenitis suppurativa. Am Fam Physician. May 1 2005;71(9):1652, 1657. [Medline].

  100. Mandal A, Watson J. Experience with different treatment modules in hidradenitis suppuritiva: a study of 106 cases. Surgeon. Feb 2005;3(1):23-6. [Medline].

  101. Canoui-Poitrine F, Revuz JE, Wolkenstein P, 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.

  102. Barth JH. Cutaneous Virilism, Apocrine Glands and Hidradenitis Suppurativa [thesis]. London: University of London; 1992.

  103. Fitzsimmons JS, Guilbert PR, Fitzsimmons EM. Evidence of genetic factors in hidradenitis suppurativa. Br J Dermatol. Jul 1985;113(1):1-8. [Medline].

  104. Gao M, Wang PG, Cui Y, et al. Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3. J Invest Dermatol. Jun 2006;126(6):1302-6. [Medline].

  105. Giamarellos-Bourboulis EJ, Antonopoulou A, Petropoulou C, et al. Altered innate and adaptive immune responses in patients with hidradenitis suppurativa. Br J Dermatol. Jan 2007;156(1):51-6. [Medline].

  106. Jemec GB, Gniadecka M. Sebum excretion in hidradenitis suppurativa. Dermatology. 1997;194(4):325-8. [Medline].

  107. Lapins J, Asman B, Gustafsson A, Bergstrom K, Emtestam L. Neutrophil-related host response in hidradenitis suppurativa: a pilot study in patients with inactive disease. Acta Derm Venereol. May 2001;81(2):96-9. [Medline].

  108. Lapins J, Olerup O, Emtestam L. No human leukocyte antigen-A, -B or -DR association in Swedish patients with hidradenitis suppurativa. Acta Derm Venereol. Jan-Feb 2001;81(1):28-30. [Medline].

  109. Lewis F, Messenger AG, Wales JK. Hidradenitis suppurativa as a presenting feature of premature adrenarche. Br J Dermatol. Oct 1993;129(4):447-8. [Medline].

  110. Matusiak L, Bieniek A, Szepietowski JC. Hidradenitis suppurativa and associated factors: still unsolved problems. J Am Acad Dermatol. Aug 2009;61(2):362-5. [Medline].

  111. Montgomery JR, White TW, Martin BL, Turner ML, Holland SM. A novel connexin 26 gene mutation associated with features of the keratitis-ichthyosis-deafness syndrome and the follicular occlusion triad. J Am Acad Dermatol. Sep 2004;51(3):377-82. [Medline].

  112. Mowad CM, McGinley KJ, Foglia A, Leyden JJ. The role of extracellular polysaccharide substance produced by Staphylococcus epidermidis in miliaria. J Am Acad Dermatol. Nov 1995;33(5 Pt 1):729-33. [Medline].

  113. Pavlovic M. Oboljenja apokrinih znojnih zlezda. In: Karadaglic D, ed. Dermatologija Beograd: Vojnoizdavacki Zavod. 2000:754-61.

  114. Shelley WB, Cahn MM. The pathogenesis of hidradenitis suppurativa in man; experimental and histologic observations. AMA Arch Derm. Dec 1955;72(6):562-5. [Medline].

  115. Wasik F, Barancewicz-Losek M. Hryncewicz-Gwozdz A, Jelen M: Hidradenitis suppurativa complicated by hidradenocarcinoma. Dermatol Klin (Wroclaw). 2001;3 (Suppl 1):64.

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

hidradenitis suppurativa, HS, acne inversa, acne triad, acne tetrad, hidradenitis axillaris, apocrinitis, intertriginous acne, pyoderma fistulans sinifica, Verneuil's disease, Verneuil disease, dissecting cellulitis of scalp and neck, acne conglobata, follicular occlusion triad, follicular occlusion tetrad, pilonidal sinus, acneiform disorder, apocrine occlusion

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Marina Jovanovic, MD, PhD, Chief of Dermatology Ward and Contact Dermatitis Investigative Unit, Clinic of Dermatoveneroleogic Diseases, Clinical Center, Novi Sad, Serbia; Professor in Dermatology, Medical Faculty, University of Novi Sad, Vojvodina, Serbia
Disclosure: Nothing to disclose.

Coauthor(s)

George Kihiczak, MD, Clinical Associate Professor, Department of Dermatology, New Jersey Medical School and University Hospital
George Kihiczak, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Medical Society of New Jersey
Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.