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 and Montenegro; Associate Professor in Dermatology, Medical Faculty, University of Novi Sad, Vojvodina, Serbia and Montenegr
Coauthor(s): George Kihiczak, MD, Clinical Associate Professor, Department of Dermatology, New Jersey Medical School University Hospital; Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Jan 24, 2007

Introduction

Background

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

Pathophysiology

HS has traditionally been considered a disorder of the apocrine glands. HS was first described as a distinct entity in 1839, when Velpeau1 reported a patient with the superficial abscess formation in the axillary, mammary, and perianal regions. In 1854, Verneuil2 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 HS. Verneuil did not perform any histopathologic studies, and he conceded that his conclusions were based purely on the characteristic distribution of the condition.

In 1922, Schiefferdecker3 classified the sweat glands as eccrine and apocrine, and he subsequently localized HS to the apocrine glands. In 1939, Brunsting4 provided a detailed description of the histologic features of HS. 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, Brunsting4 highlighted its frequent association with acne. He noted that HS, dissecting cellulitis of the scalp and 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.

In 1956, Pillsbury et al5 combined acne conglobata, HS, and dissecting cellulitis under the term follicular occlusion triad. The only flaw in their concept was the focus on apocrine sweat gland involvement. In 1975, Plewig and Kligman6 added pilonidal sinus as another component to the ensemble, and they introduced the term acne tetrad. Plewig and Kligman pointed out that HS is a misnomer because of the lack of apocrine gland involvement, but they did not present a detailed explanation. In 1989, Plewig and Steger7 suggested the term acne inversa as an inclusive and accurate name for what was previously called the follicular occlusion triad, or follicular occlusion tetrad. Eventually, HS was accepted as an acneiform disorder that begins with follicular occlusion rather than an infection of the sweat glands.

Because HS is actually a defect of follicular epithelium, some authorities have suggested excluding all outdated synonyms for this disease, including HS, and substituting the term acne inversa. The term acne inversa links the pathogenesis to acne and reflects the fact that the condition is an expression of follicular occlusion in localizations inverse to acne vulgaris.

Frequency

United States

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

International

The prevalence of HS appears to be 1% of the general population; it was 4% in a group of young adults who were treated at a clinic for sexually transmitted diseases. HS is probably more common than once thought, but the diagnosis is frequently ignored or missed. HS 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 HS. HS is a chronic disabling disorder that relentlessly progresses and leads to keloids, contractures, and immobility. Patients can become outcasts or at least have difficulty making social contact.

  • In an assessment of socioeconomic effects, Jemec et al8 noted that the most common problem in both sexes was soreness and that the average patient lost 2.7 days of work per year. In comparison, in Denmark, the average number of work days 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 HS entails less morbidity than perhaps hand eczema, with regard to its prevalence, it significantly contributes to the average number of work days lost (Danish National Statistical Office, Copenhagen, Denmark, Jemec GB, written communication, 1996).
  • Jemec et al8 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 other contributing factors. 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.
  • The general self-reported level of health, which is well correlated with more objective parameters of morbidity, is significantly worse among HS patients than among healthy control subjects.
  • The mean Dermatology Life Quality Index score for HS is higher than for previously studied skin diseases and correlated with disease intensity as expressed by lesions per month. This suggests that the Dermatology Life Quality Index score may be a relevant outcome measure in future therapeutic trials in HS patients.

Race

  • Although many authors report no racial predilection, an increased frequency is observed in blacks, possibly because blacks have a greater density of apocrine glands than whites.

Sex

  • Although HS is widely considered to occur more frequently in females than in males, with a ratio as high as 2-5:1, reports on sex prevalence are controversial.
  • Active genitofemoral lesions occur significantly more often in female patients, whereas perianal involvement tends to be more common in men. No sex difference is seen in the axillary lesions. Large multiheaded comedones are characteristic findings of the disease. Comedones have been suggested as precursor lesions for HS. They appear to be equally distributed in both sexes and sites.

Age

  • The onset of HS peaks in individuals aged 11-50 years, with an average patient age of 23 years.
  • In less than 2% of patients, the disease appears before age 11 years.
  • In extremely rare cases, HS occurs before puberty or after menopause.

Clinical

History

HS usually occurs in otherwise healthy individuals, and it rarely begins before puberty.

  • The disease onset is insidious, with early symptoms of pruritus, erythema, and local hyperhidrosis.
  • Later, the lesions become painful.
  • Arthropathy associated with HS may manifest with variable clinical features, ranging from asymmetric pauciarticular arthritis to a symmetric polyarthritis and/or polyarthralgia syndrome.

Physical

The diagnosis is primarily clinical, and biopsy is rarely required, especially in well-developed lesions. Recently, the consensus approach deemed that 3 key elements are required to diagnose HS: 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 HS: the axillae and the groin. These areas are defined by anatomical borders and are called designated sites. HS is diagnosed if the patient has either (1) 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 or (2) 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 lesions

Physical findings are as follows.

  • HS has a predilection for the intertriginous regions.
    • The axillary and inguinoperineal regions are most commonly affected (see Media Files 1-2).
    • 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.
  • The extent and the severity of the disorder vary widely.
    • Some patients have relatively mild forms of the disease that involve only one region.
    • 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 spreading to the scrotum, the labia, the mons pubis, the mammary or perianal region, and the buttocks.
  • A firm pea-sized nodule can appear and may spontaneously rupture, yielding a purulent discharge. The lesion then heals, with fibrosis and eventual recurrence in the 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 HS; these sinuses periodically discharge 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 Media Files 3-4).
    • 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.
    • Over time, multiple abscesses and sinus tracts form a subcutaneous honeycomb.
    • Occasionally, the involvement extends into the underlying fascia and muscles.
    • Ultimately, fibrosis, hypertrophic scarring, and induration develop.
  • Multiple open comedones and so-called bridged comedones are the hallmark finding of HS; 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 fistulas.
    • In the upper anal fold, terminal hairs emerge from thickened scars.
    • Perianal HS 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 HS may be clinically identical to the cutaneous manifestations of Crohn disease (CD). CD may be complicated by a variety of skin manifestations, and HS has been reported to precede or complicate CD. In examining patients with perianal HS, Church et al9 noted that CD coexisted with perianal HS in 39% of the patients.
    • Local swelling and inflammation associated with CD may precipitate HS in patients already prone to it. However, the coexistence of CD and HS does not explain the frequent presence of axillary, groin, and buttock involvement, which may imply a constitutional or genetic predisposition to HS in patients with rectal CD.
    • The coexistence of the 2 conditions may have implications in the treatment of perianal sepsis in such patients. Each condition can mask the other. HS may adversely affect the clinical course of patients with CD. Furthermore, patients with both conditions are more prone to persistent sepsis and frequently require proctocolectomy and fecal diversion procedures.
    • In patients with the chronic lesions due to so-called granulomatous HS, as well as in 60% of patients with CD, the presence of noncaseating epithelioid-type granulomas suggests that HS and CD are chronic inflammatory conditions. Epithelioid granulomas occur in patients with a constitutive tendency to form granulomas as a part of an immunologic abnormality.
    • Whether the association between HS and CD is more than a coincidence is unclear; however, the small proportion of patients with HS and epithelioid granulomatous inflammation may represent a group that is susceptible to systemic granulomatous disease, including CD.
  • The association of HS with several disorders in which poral occlusion is prominent supports the theory of a follicular origin of HS.
    • Such disorders include Fox-Fordyce disease, acanthosis nigricans, pityriasis rubra pilaris (PRP), steatocystoma multiplex, and Dowling-Degos disease.
    • HIV-associated PRP, or PRP type VI, is a new entity reported in patients with HIV disease. HIV-associated PRP is characterized by the cutaneous lesions of PRP and a variable association with the lesions of acne conglobata, HS, and lichen spinulosus. This disease can be designated by the wider term HIV-associated follicular syndrome. Although the pathogenesis of PRP type VI is unknown, follicular inflammation secondary to HIV infection of the hair follicle bulge area has been suggested.
  • 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 HS, acne conglobata, perifolliculitis abscedens, and suffodiens capitis. Arthritis associated with acne fulminans, seronegative spondyloarthropathies of psoriatic arthritis, and Reiter disease are well-defined clinical entities. However, arthritis associated with HS, acne conglobata, perifolliculitis abscedens, suffodiens capitis, and SAPHO (synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome are less well defined; these conditions may be part of the spectrum of HLA-B27–negative spondyloarthropathies.
    • The pathogenesis of the arthritis remains unknown but may include an inappropriate response to bacterial antigens involved in acne or some other autoimmune response.
    • Arthropathy associated with HS may manifest with variable clinical features, ranging from 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 also may be involved.
    • In many instances, the arthropathy worsens during flares of HS, and, conversely, it improves after HS resolves.
    • The concept of SAPHO syndrome includes 4 subgroups of osteoarticular disorders: (1) rheumatologic manifestations associated with acne conglobata or acne fulminans or inversa-HS, (2) pustulosis palmoplantaris, (3) sternocostoclavicular hyperostosis, and (4) chronic multifocal recurrent osteomyelitis.
    • SAPHO syndrome may encompass cases described as arthropathy associated with HS 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 HS and acne conglobata.
    • Recent reports raised the question of whether mammillary fistula or Zuska disease might be a manifestation of acne inversa.
    • Pyoderma gangrenosum has been rarely associated with HS and has occurred only after HS has been present for at least 2 decades, as can be seen in the authors' patient (see Media Files 5-15). Only 15 cases of coexistent pyoderma gangrenosum and HS were found in the English-language literature. More studies are required to support impaired neutrophil function as a common etiological pathway.

Causes

The exact etiology of HS remains obscure. All proposed etiologic factors, such as occlusion and bacterial infection, genetics, host defense defects, hormones, cigarette smoking, and irritants, are likely only secondary factors. The primary events in the hair follicles of the affected areas remain unidentified. In addition, thickened skin may play a role in the pathogenesis of HS (see Imaging Studies).

  • Classic HS is an occlusive and pyogenic disease of the apocrine glands, a hypothesis that seemed to be confirmed with its experimental reproduction by Shelley and Cahn in 1955.10 After manually depilating the skin and applying atropine-impregnated tape, they induced initial keratinous obstruction, dilatation, and inflammation of the apocrine duct, which occurred in only 25% of the experimental lesions. No progression to the characteristically chronic condition of HS occurred.
    • In more recent studies, HS is identified as a disorder of follicular occlusion rather than apocrine occlusion. Yu and Cook11 found inflammatory changes involving the apocrine glands in only one third of cases; these occurred only when the inflammation extensively involved the hair follicles and eccrine glands. Attanoos et al12 reported follicular occlusion in all specimens, when compared with controls, regardless of disease duration. The inflammation of the apocrine glands did not occur in the absence of an adjacent folliculitis; thus, apocrine gland involvement was incidental or secondary. Therefore, HS is best considered a disorder of the terminal follicular epithelium in the apocrine gland–bearing skin.
    • The earliest change is plugging, which occurs in follicular hyperkeratosis with infundibulofolliculitis. This obstructs the apocrine gland ducts and promotes perifolliculitis around the ducts. Whether this initial inflammatory change is due to a bacterial infection or factors similar to those involved in acne formation is not known.
    • In the later stages of HS, bacterial infection seems to be a risk factor for the destructive scarring and extension of HS lesions, and, once the sinuses have formed, the risk of secondary infection is obvious.
    • Among the most commonly isolated bacteria are coagulase-negative staphylococci. Sweat ducts can become occluded with periodic acid-Schiff (PAS)–positive extracellular polysaccharide substance, the cause of which was recently suggested to be Staphylococcus epidermidis infection. Such strains induced miliaria under experimental conditions. A similar mechanism may be important in the pathogenesis of HS.
    • The earliest inflammatory event in HS is the rupture of the follicular epithelium. The cause of the rupture is not known, although friction in intertriginous locations may be a contributing factor. The rupture is followed by the spillage of foreign-body material into the dermis, which initiates an inflammatory response, resulting in foreign-body granuloma formation. Epithelial strands form draining sinuses in this inflammatory tissue. Colonization with bacteria, usually coagulase-negative staphylococci, can aggravate the chronic inflammation.
  • Although the inciting influences for the follicular occlusion and sinus tract formation have not been fully elucidated, genetic factors may play a role.
    • More than 15 years ago, the existence of a familial form of HS with autosomal dominant inheritance was proposed. The disease frequency among first-degree relatives of patients with familial HS was 34%. However, this finding does not seem to be widely accepted; why this finding is not accepted is unclear because it had repeatedly been recognized that a number of patients with HS have affected family members.
    • Findings from a recent review of the original study group supported the previous results, although conclusive proof is still lacking.
    • A search for the molecular genetic abnormality is now necessary. Mutation within the connexin 26 gene was recently reported in association with keratitis-ichthyosis-deafness syndrome and with the severe follicular occlusion triad. The notion that specific HLA antigens might be at the center of a genetic susceptibility to HS is not supported and remains questionable.
  • Host-defense defects in patients with HS are suspected but not proven.
    • Hyperreactive neutrophils have been proposed to be of pathophysiologic importance in many chronic inflammatory diseases involving the destruction of the surrounding tissue by the simultaneous release of reactive oxygen species and active proteases.
    • The release of oxygen radicals from peripheral neutrophils that are activated in vitro was studied in patients with inactive HS. The generation of free oxygen radicals after the stimulation of peripheral neutrophils with protein kinase C activator and phorbolmyristate acetate was significantly higher in patients with HS than in healthy control subjects.
    • The higher sensitivity of protein kinase C to phorbolmyristate acetate in patients with HS is unlikely to have been induced by the disease and its local lesions because the systemic effects were minor in the quiescent state. Therefore, a defect in the function of the neutrophils might be of pathogenic importance in HS.
  • Apocrine sweat glands are stimulated by androgen and suppressed by estrogen. Evidence is documented for hormonal effects in HS; however, the exact roles of androgens in the pathogenesis of HS remain controversial, and they may prove to be secondary.
    • Many women describe a worsening of the condition with menses, whereas others report alleviation with pregnancy, followed by postmenstrual flaring. These observations suggest that the low level of estrogen predisposes women to disease activity. Recently, premenstrual flares were shown to be unrelated to menstrual disturbances. These flares were not predictive of the overall course, and the effect of pregnancy was not constant. The female preponderance may also be explained by other specific factors, such as the estrogenic influence on inflammation.
    • The following evidence supports the association of androgens and HS: the disease is rarely present until after puberty begins, HS is not present in eunuchs or eunuchoids, and HS may occur as the presenting feature of premature adrenarche. In addition, antiandrogen therapy is of some benefit in patients with HS.
    • The relationship between HS and hyperandrogenism is largely based on the finding that the free androgen index is increased due to a low level of sex hormone–binding globulin (SHBG). SHBG is now believed to be regulated by factors that influence body weight.
    • Hirsutism and obesity are common findings among women with HS.

      • Obesity can alter sex hormone metabolism, leading to an androgen-excess state. Excessive androgens can enhance keratin production and coarsening of the hair shaft, promoting follicular occlusion.
      • Although hirsutism, obesity, and acne are more common than expected among women with HS, the prevalence rates are not significantly different from those in the general population. However, neither evidence for biochemical hyperandrogenism nor suppression of SHBG has been shown in women with HS compared with age-, body weight–, and hirsuties-matched controls.
      • In one study, obesity and oral contraceptive use were not common or pronounced among patients; this observation suggested that while these parameters may influence preexisting disease, they are unlikely to be pathogenically important.
      • In obesity, increased skin-to-skin contact may promote follicular hyperkeratosis.
    • An abnormal end-organ response to normal circulating levels of androgens is proposed. The normal apocrine gland contains 5-alpha reductase, which converts testosterone to the potent androgen dihydrotestosterone. Finasteride is a competitive inhibitor of the 5-alpha reductase type II isoenzyme. The benefits of finasteride in some patients with recalcitrant and persistent forms of HS raised the issue of whether 5-alpha reductase type I or type II is expressed in this disease and whether this expression applies to the apocrine gland, sebaceous gland, or both. On the other hand, sebum excretion is not an important factor in the development of HS. Thus, hormonal influence remains controversial.
  • Cigarette smoking may be among the major triggering factors in persons with HS, and its cessation should be encouraged. However, whether cessation improves the course of disease is unknown. Precisely what pathogenetic mechanism is responsible for the effect of smoking on the manifestations of acne inversa remains unclear, but an altered chemotaxis of polymorphic neutrophils could play a role.
  • Chemical irritants (eg, deodorants) and mechanical irritation (eg, depilation, shaving) have been considered risk factors. However, in one study, no significant difference was found in patients who were exposed to these factors compared with age-matched control subjects. Factors associated with disease activity, such as heat, sweating, stress, and menstruation in females, are the most commonly cited factors that exacerbate the disease. In one study, 32% of responders observed deterioration of their disease during the summer.
  • HS is rarely an adverse effect of drug use, but oral contraceptive use and lithium use have been associated with its development.
  • Lung and buccal cancer are more common among HS patients than in the general population, as would be expected with increased tobacco smoking and chewing.
  • As reported by Wasik et al in 2001,13 HS may rarely be complicated by hidradenocarcinoma.

More on Hidradenitis Suppurativa

Overview: Hidradenitis Suppurativa
Differential Diagnoses & Workup: Hidradenitis Suppurativa
Treatment & Medication: Hidradenitis Suppurativa
Follow-up: Hidradenitis Suppurativa
Multimedia: Hidradenitis Suppurativa
References
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References

  1. Velpeau A. Dictionnaire de Medicine, un Repertoire des Sciences Medicales sons le Rapport, Theorique et Pratique. 2nd ed. Paris, France: 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, Germany: 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, Pa: WB Saunders; 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. Acne and Related Disorders. London, England: Martin Dunitz Ltd; 1989:343-57.

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

  9. 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].

  10. Shelley WB, Cahn MM. The pathogenesis of hidradenitis suppurativa in man. Arch Dermatol Syphilol. 1955;72:562-5.

  11. 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].

  12. 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].

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

  14. 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].

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

  16. 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].

  17. 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].

  18. Asselineau D, Darmon M. Retinoic acid provokes metaplasia of epithelium formed in vitro by adult human epidermal keratinocytes. Differentiation. Apr 1995;58(4):297-306. [Medline].

  19. Baran E, Maj J, Nockowski P, Kibler-Nockowska M. Pyoderma gangrenosum in the course of hidradenitis suppurativa. Dermatol Klin (Wroclaw). 2001;3 (Suppl 1):188.

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

  21. 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].

  22. 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].

  23. 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].

  24. 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].

  25. Boer J, Weltevreden EF. Hidradenitis suppurativa or acne inversa. A clinicopathological study of early lesions. Br J Dermatol. Nov 1996;135(5):721-5. [Medline].

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

  27. 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].

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

  29. Champion RH. Disorder of sweat glands. In: Rook A, Wilkinson DS, Ebling FJ. Textbook of Dermatology. 6th ed. Oxford, England: Blackwell Science; 1998:1985-2002.

  30. 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].

  31. 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].

  32. Daoud MS, Dicken CH. Apocrine glands. In: Freedberg IM, Eisen AZ, Wolff K, et al. Fitzpatrick's Dermatology in General Medicine. 5th ed. New York, NY: McGraw-Hill; 1999:1999:810-17.

  33. 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].

  34. Endo Y, Tamura A, Ishikawa O, Miyachi Y. Perianal hidradenitis suppurativa: early surgical treatment gives good results in chronic or recurrent cases. Br J Dermatol. Nov 1998;139(5):906-10. [Medline].

  35. 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].

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

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

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

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

  40. 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].

  41. 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].

  42. Gold MH. Aminolevulinic acid photodynamic therapy for hidradenitis suppurativa. Dermatol Clin. Jan 2007;25(1):67-73. [Medline].

  43. González-López 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].

  44. 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].

  45. 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].

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

  47. Hurley HJ. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa and familial benign pemphigus: Surgical approach. In: Roenigk RA, Roenigk HH Jr. Dermatologic Surgery, Principles and Practice. New York, NY: Marcel Dekker; 1989:717-45.

  48. 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].

  49. 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].

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

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

  52. 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].

  53. 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].

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

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

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

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

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

  59. 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].

  60. 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].

  61. Kleeman D, Trüeb 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].

  62. Lapins J, Asman B, Gustafsson A, Bergström 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].

  63. 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].

  64. 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].

  65. 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].

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

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

  68. 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].

  69. 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].

  70. 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].

  71. 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].

  72. 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].

  73. 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].

  74. 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].

  75. 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].

  76. 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].

  77. 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].

  78. 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].

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

  80. Papadopoulos AJ, Kihiczak G, Schwartz RA. Hidradenitis suppurative: an update. Acta Derm Venerol (Ljubljana). 2000;9:131-35.

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

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

  83. 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].

  84. 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].

  85. 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].

  86. 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].

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

  88. Sartorius K, Lapins J, Emtestam L, Jemec GB. Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. Br J Dermatol. Jul 2003;149(1):211-3. [Medline].

  89. Sartorius K, Lapins J, Jalal S, Emtestam L, Hedberg M. Bacteraemia in patients with hidradenitis suppurativa undergoing carbon dioxide laser surgery: detection and quantification of bacteria by lysis-filtration. Dermatology. 2006;213(4):305-12. [Medline].

  90. 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].

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

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

  93. 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].

  94. Thein M, Hogarth MB, Acland K. Seronegative arthritis associated with the follicular occlusion triad. Clin Exp Dermatol. Sep 2004;29(5):550-2. [Medline].

  95. Thielen AM, Barde C, Saurat JH. Long-term infliximab for severe hidradenitis suppurativa. Br J Dermatol. Nov 2006;155(5):1105-7. [Medline].

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

  97. Troyanovsky SM, Guelstein VI, Tchipysheva TA, Krutovskikh VA, Bannikov GA. Patterns of expression of keratin 17 in human epithelia: dependency on cell position. J Cell Sci. Jul 1989;93 ( Pt 3):419-26. [Medline].

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

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

  100. Winterfield LS, Menter A. Infliximab. Dermatol Ther. 2004;17(5):409-26. [Medline].

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

  102. Wolkenstein P, Loundou A, Barrau K, Auquier P, Revuz J,. Quality of life impairment in hidradenitis suppurativa: a study of 61 cases. J Am Acad Dermatol. Apr 2007;56(4):621-3. [Medline].

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Further Reading

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Keywords

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

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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 and Montenegro; Associate Professor in Dermatology, Medical Faculty, University of Novi Sad, Vojvodina, Serbia and Montenegr
Disclosure: Nothing to disclose.

Coauthor(s)

George Kihiczak, MD, Clinical Associate Professor, Department of Dermatology, New Jersey Medical School 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 of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of 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 Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Daniel Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American Academy of Facial Plastic and Reconstructive Surgery, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for Laser Medicine and 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; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White 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, Department of Dermatology, Penn State University, Milton S Hershey Medical Center
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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.

 
 
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