eMedicine Specialties > General Surgery > Glands

Hidradenitis Suppurativa

Naveen Pokala, MBBS, MS, FRCS, Staff Physician, Department of Surgery, Bronx Lebanon Hospital
Ravi Pokala Kiran, MBBS, MS, FRCS (Eng), FRCS (Glas), Staff Physician, Department of General Surgery, St Mary's Hospital; Suhasini Yadlapati, MBBS, Staff Physician, Department of Surgery, Gandhi General Hospital, India; Ajay K Shah, MD, Consulting Surgeon, Department of Surgery, Bronx-Lebanon Hospital Center Healthcare System

Updated: Apr 2, 2008

Introduction

Hidradenitis suppurativa is a chronic, relapsing, suppurative cicatrizing disease occurring in the apocrine follicles. The disease has a propensity to become chronic and indolent because of subcutaneous extension leading to induration, sinus, and fistula formation.

History of the Procedure

Leper provided the first descriptions of hidradenitis suppurativa in 1839, when he noticed peculiar localization of abscesses within the axillary and the perianal skin. Verneuil first described the apocrine gland involvement in 1854.¹

Clinical features of the disease were described by Lane and Brunstig.² Experimental reproduction was achieved by Shelly and Cahn in 1955, which helped establish the pathogenesis of the disorder.³ Further work by Conway, Paletta, Pollock, Letterman, and others in establishing the surgical management of the disease has been noteworthy.^4,5,6,7

Frequency

In the US: Hidradenitis suppurativa occurs more commonly in women than in men and usually occurs in the third decade of life. One study by Brown et al determined the prevalence in industrialized countries to be 0.3-4%.⁸

Internationally: The prevalence has not been accurately determined. The disease is found more commonly in the white population and the black population and is rarely observed in the Asian population.

Age: Hidradenitis suppurative usually begins in the postpubertal age group, when the apocrine glands start developing. It is most common in the third decade of life, but the untreated disease may persist into the seventh decade.

Sex: The condition is more common in women than in men. Submammary, axillary, and inguinal involvement is more common in females, whereas the perineal form is more common in men.

Site: Hidradenitis suppurative is found in the following areas:

• Skin-bearing apocrine glands
• Axilla
• Groin
• Perineum
• Perianal region
• Buttocks
• Scrotum
• Submammary region

Etiology

The exact cause of hidradenitis suppurativa has not been determined, although the following theories have been proposed:

• Folliculitis is observed in all patients with hidradenitis suppurativa; whether this is coincidental or causative has not been established.
• Local frictional trauma has been proposed to be one of the causative factors.
• Infective etiology
  • Streptococci, staphylococci, and Escherichia coli have been identified in the early stages of the disease; however, in the chronic relapsing stages, anaerobic bacteria and Proteus species have more commonly been isolated.
  • Whether the bacteria are the cause or the result of the disease has not been determined.
• Diabetes, impaired glucose intolerance, and obesity were observed in some patients with hidradenitis suppurativa. Studies have shown that these entities are only incidental findings and not causative.
• Hormonal theory: Improvement and relapse after pregnancy and contraceptive pill intake suggest that low levels of estrogens cause a predisposition for hidradenitis suppurativa.
• Immune theory: Immunity in most patients is intact, but some patients demonstrate a defect in the T-cell lymphocytes.
• Genetic theory: Increased incidence in individuals with HLA-A1 and HLA-B8 has been demonstrated in some patients.
• Association tetrads: Hidradenitis suppurativa is part of the tetrad of acne conglobata (cystic acne), pilonidal sinus, and perifollicular capitis.
• Cigarette smoking and lithium therapy have been identified as triggering factors for the disease.

Pathophysiology

In the initial predestructive stages, keratin comedones are observed in the apocrine gland follicles, along with inflammatory cells around the apocrine glands and distended ducts filled with leucocytes and secretions. Later, groups of cocci may be observed within the gland and in the dermis, indicating infection and abscess formation. Some evidence suggests that the occlusion of abnormal hair follicles may lead to the initiation of these changes.

The suppuration later extends into the adjacent and subcutaneous tissue, where there may be chronic inflammatory cells involving histiocytes and giant cells around the apocrine gland remnants and the keratin plugs.

The chronic process leads to dense fibrosis and sinus and fistulous tracts lined partly by granulation tissue and partly by squamous epithelium.

The Pathogenesis of Hidradenitis Suppurativa

Presentation

The onset of disease is usually after puberty, in the second and third decades, when the apocrine sweat glands start developing. It begins in the epithelium containing apocrine and is more common in the neck, the axilla, and the submammary region in females and in the perineal skin in males. The perineal disease tends to be more severe.

The early lesions are solitary, painful pruritic nodules that may persist for weeks or months without any change. If subcutaneous extension occurs, it may appear as indurated plaques, which, in lax skin, such as the axilla and the groin, manifest as linear bands. Multiple sites may be simultaneously affected.

The nodules develop into pustules and eventually rupture externally, draining purulent material. Healing occurs with dense fibrosis, and recurrences crop up in and around the original site. This leads to chronic sinus formation, with intermittent release of serous, purulent, or bloodstained discharge. Ulceration sometimes occurs, and the abscesses may burrow and rupture into the neighboring structures. Episodes of acute cellulitis are sometimes a feature and are accompanied by fever and toxicity.

Regional lymphadenopathy is characteristically absent. Chronic axillary hidradenitis suppurativa usually causes a reduction of the normal axillary odor.

Severity and course of the disease are variable, but untreated hidradenitis suppurativa is typically a relentless progressive disease with acute exacerbations and remissions that lead to sinus tract formation and marked scarring.

Differential diagnoses include the following conditions:

• Infected cystic acne
• Lymphogranuloma venereum
• Developmental fistulae
• Crohn disease
• Furunculosis
• Scrofuloderma
• Actinomyces

Comedones should be scrutinized; the nonspecific histologic changes may help in diagnosis.

Indications

Recurrent abscess formation and formation of chronic sinus tracts with recurrence are the usual indications for surgery.

Relevant Anatomy

The anatomy is related to the affected site (eg, axilla, perineum, inguinal or perianal region). Adequate attention must be paid to the vessels and the nerves running through the respective regions. An attempt must be made to preserve these important structures during the dissection.

Contraindications

The acute abscess stage is a relative contraindication for curative surgery, which can be performed subsequent to a short course of antibiotic therapy.

Workup

Laboratory Studies

• Blood tests
  • A complete blood cell count identifies the underlying anemia associated with the chronic disease.
  • Blood sugar tests identify associated diabetes.
  • Cell-mediated and immune response studies and T- and B-cell assays usually return normal findings.
  • Routine endocrine assays identify the hormonal imbalances.
  • Routine biochemistry for preoperative workup is essential.
• Microbiology
  • In the acute stages, the isolated organisms include the coagulase negative staphylococci, E coli, Streptococcus milleri, and the anaerobic Bacteroides species. Proteus species have been isolated from patients with chronic conditions.
  • Staphylococci have been shown to be the transient bacteria in the acute initial stages, whereas S milleri is the predominant organism in the chronic stages.

Imaging Studies

• CT scans can be useful before planning surgery because they help to accurately map the extent of disease.

Histologic Findings

Early lesions show keratinous obstruction of the distal apocrine ducts, with dilatation and rupture of the adjacent tubules. Neutrophilic infiltrate is usually found within the tubule initially; after rupture, infiltrate is found in the surrounding tissue.

Chronic disease shows lymphocytic infiltration, granulation tissue, fibrous tissue, sinus tract formation, pseudoepitheliomatous hyperplasia, and obliteration of the glandular elements as the disease progresses.

While the earlier lesions are diagnostic of hidradenitis suppurativa, the later features can also be produced by severe cystic acne and by diseases that cause chronic sinus tract formation.

Staging

The disease can be divided into the following 3 clinical stages:

• Stage 1: Single or multiple abscesses form, without sinus tracts and cicatrization.
• Stage 2: Recurrent abscesses form, with tract formation and cicatrization. There may be single or multiple widely separated lesions.
• Stage 3: Diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses are observed across the entire area.

Treatment

Medical Therapy

Treatment depends upon the stage of the disease. Early lesions are usually treated by medical therapy, whereas the patient with long-standing indolent disease requires surgical therapy.

Nonspecific treatment measures include good hygiene, weight reduction, use of antiseptic detergents, and avoidance of tight-fitting clothes.

• Acute-stage treatment options
  • Antibiotics: A short course of antibiotics for a period of 2 weeks is usually advisable. The antibiotics used include a combination of erythromycin and metronidazole, minocycline, or clindamycin. Cephalosporins and penicillins can also be used.
  • Intralesional steroids: Intralesional injection of steroids (eg, triamcinolone 5-10 mg diluted with water) causes the early lesions to involute within 12-24 hours.
• Chronic relapsing–stage treatment options
  • Long-term antibiotics: Long-term administration of erythromycin and tetracycline has been used to treat the chronic stages and is shown to reduce the relapse rate. However, the efficacy of the antibiotics may be lost after long-term use. Efficacy can usually be regained by stopping the drug for a month and restarting it.
  • High-dose systemic steroids (eg, prednisolone 60 mg/d) are useful as adjuvants to antibiotics, and they act by reducing the inflammatory process.
  • Estrogens: Contraceptive pills (eg, 50 mcg ethynyl estradiol) and the combination of estrogens with 100 mg of cyproterone acetate have been used.
  • Retinoids: These have been shown to be effective in the chronic disease. Isotretinoin, at a dose of 1 mg/kg/d, is administered for 4 months. Etretinate, at a dose of 0.5 mg/kg/d for a period of 6 months, is used for patients whose conditions are unresponsive to isotretinoin. Retinoids are teratogenic, and pregnancy is prevented by the use of contraception. Approximately 40% of patients show good response to retinoids.
• Other therapeutic agents that have been used with limited success include cisplatin, methotrexate, 5-alpha reductase inhibitors, and TNF-alpha inhibitors. Infliximab has been used in patients with Crohn disease and associated hidradenitis suppurativa.

Surgical Therapy

• Stage 1 options include incision and drainage, followed by antibiotics.
• Stage 2 and some stage 3 options include minor procedures.
  • Exteriorization and laying open of tracts and electrocoagulation
  • Excision and primary closure (eg, the Pollock procedure)
• Stage 3 options include total wide excision and healing with secondary intention or flaps and grafts.
• Closure of defects is achieved by the following:
  • Z-plasty
  • Skin grafts
    • Thiersch split-thickness grafts
    • Meshed grafts
    • Wolfe full-thickness grafts
  • Flaps
    • Rotation flaps
    • Free flaps
  • Biosynthetics agents, like Biobrane and Integra, have also been used.

Preoperative Details

• Be aware of the possibility of associated systemic abnormalities.
• Perform routine preoperative assessment for surgery and anesthesia, including cardiac, respiratory, and renal assessments.
• Order antibiotics to treat the acute exacerbations before surgery.
• Order prophylactic antibiotics before surgery.
• Warn patients of the likelihood of a large raw area, which will require prolonged postoperative dressings.

Intraoperative Details

• Perform a wide excision, with a 1.5-cm margin all around the lesion.
• Perform intraoperative mapping of the sinus tracts with methyl violet, which reduces recurrence rates.
• In the genital and perianal area, primary closure is to be avoided, and healing by secondary intention is advocated.
• Axilla: Total excision with transverse primary closure (ie, the Pollock procedure) is usually possible.

Postoperative Details

• Postoperative care
  • Regular and prolonged use of postoperative dressings is necessary to aid secondary healing.
  • Healing can be hastened by the use of silastic foam dressings.
  • The use of Betadine and chlorhexidine dressings and compression has been shown to be of benefit.
• Postoperative complications
  • Patients can develop general complications, such as pneumonia, deep venous thrombosis (DVT), and infection. Administer routine perioperative DVT and antibiotic prophylaxis.
  • Active physiotherapy, breathing exercises, and early ambulation are encouraged.
  • Postoperative complications specific to the procedure include wound breakdown, hematoma formation, wound infection, and graft rejection and failure.
  • The overall complication rate is 17-20%.

Follow-up

Patients are monitored in the clinic at regular intervals for at least 6 months before they can be declared to be cured.

Complications

The complications of long-standing untreated disease include the following:

• Fistula formation into the urethra, bladder, rectum, or peritoneum has been reported.
• Sequelae of chronic infection, such as secondary anemia, hypoproteinemia, amyloidosis, and renal disease, can occur in chronic disease.
• Peripheral and axial arthropathy is possible.
• Chronic malaise and depression are observed.
• Scarring of the tissue can lead to lymphatic obstruction and lymphedema of the limbs and arthropathy.
• Marjolin ulcer (squamous cell carcinoma) has been reported in long-standing chronic disease.
• Scrotal elephantiasis has been reported.

Outcome and Prognosis

Locoregional recurrence does arise, and reported rates vary by 30-50%. One series reports a 100% recurrence rate with incision and drainage alone, a 42% recurrence rate with limited excision, and a 27% recurrence rate after radical excision.

Reassessment for several months in the follow-up clinic is required before cure can be assumed with certainty.

Future and Controversies

The role of radiotherapy in the treatment of the disease is controversial. One series by Frohlich et al in Germany reports complete relief in 38% of patients and improvement in 40% of patients, with 2 patients not showing any response to therapy.⁹ No complications were associated with radiotherapy.

Other series have demonstrated that radiotherapy is of no benefit; therefore, the role of radiotherapy as an adjunct is still debated.

Laser therapy has been used for the treatment of hidradenitis suppurativa.

References

1. Verneuil AS. Etudes sur les tumor de la peau. Arch Gen Med. 1854;94:693.

2. Lane JC. hidrosdenitis axillare of Verneuil. Arch Dermatol Sphilol. 1939;39:108.

3. Shelly WB, Cahn. Pathogenesis of hidradenitis suppurativa in man. Experimental and histological observations. Arch Dermatol. 1955;72:562.

4. Conway H, Stark RB, Climo S, Weeter JC, Garcia FA. The surgical treatment of chronic hidradenitis suppurativa. Surg Gynecol Obstet. Oct 1952;95(4):455-64. [Medline].

5. Paletta FX. Hidradenitis suppurativa: pathologic study and use of skin flaps. Plast Reconstr Surg. Apr 1963;31:307-15. [Medline].

6. Pollock WJ, Virnelli FR, Ryan RF. Axillary hidradenitis suppurativa. A simple and effective surgical technique. Plast Reconstr Surg. Jan 1972;49(1):22-7. [Medline].

7. Letterman G, Schurter M. Surgical treatment of hyperhidrosis and chronic hidradenitis suppurativa. J Invest Dermatol. Jul 1974;63(1):174-82. [Medline].

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

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

10. Alexander SJ. Squamous cell carcinoma in chronic hydradenitis suppurativa: a case report. Cancer. Feb 1979;43(2):745-8. [Medline].

11. 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].

12. Bell BA, Ellis H. Hydradenitis suppurativa. J R Soc Med. Jul 1978;71(7):511-5. [Medline].

13. Bhalla R, Sequeira W. Arthritis associated with hidradenitis suppurativa. Ann Rheum Dis. Jan 1994;53(1):64-6. [Medline].

14. Brook I, Frazier EH. Aerobic and anaerobic microbiology of axillary hidradenitis suppurativa. J Med Microbiol. Jan 1999;48(1):103-5. [Medline].

15. Dvorak VC, Root RK, MacGregor RR. Host-defense mechanisms in hidradenitis suppurativa. Arch Dermatol. Apr 1977;113(4):450-3. [Medline].

16. Finley EM, Ratz JL. Treatment of hidradenitis suppurativa with carbon dioxide laser excision and second-intention healing. J Am Acad Dermatol. Mar 1996;34(3):465-9. [Medline].

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

18. Harrison BJ, Kumar S, Read GF, Edwards CA, Scanlon MF, Hughes LE. Hidradenitis suppurativa: evidence for an endocrine abnormality. Br J Surg. Dec 1985;72(12):1002-4. [Medline].

19. Highet AS, Warren RE, Weekes AJ. Bacteriology and antibiotic treatment of perineal suppurative hidradenitis. Arch Dermatol. Jul 1988;124(7):1047-51. [Medline].

20. Hogan DJ, Light MJ. Successful treatment of hidradenitis suppurativa with acitretin. J Am Acad Dermatol. Aug 1988;19(2 Pt 1):355-6. [Medline].

21. Jemec GB. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. Oct 1999;41(4):658. [Medline].

22. Jemec GB. The symptomatology of hidradenitis suppurativa in women. Br J Dermatol. Sep 1988;119(3):345-50. [Medline].

23. Jemec GB, Faber M, Gutschik E, Wendelboe P. The bacteriology of hidradenitis suppurativa. Dermatology. 1996;193(3):203-6. [Medline].

24. 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].

25. Joseph MA, Jayaseelan E, Ganapathi B, Stephen J. Hidradenitis suppurativa treated with finasteride. J Dermatolog Treat. Apr 2005;16(2):75-8. [Medline].

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

27. König A, Lehmann C, Rompel R, Happle R. Cigarette smoking as a triggering factor of hidradenitis suppurativa. Dermatology. 1999;198(3):261-4. [Medline].

28. 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].

29. Melkun ET, Few JW. The use of biosynthetic skin substitute (Biobrane) for axillary reconstruction after surgical excision for hidradenitis suppurativa. Plast Reconstr Surg. Apr 15 2005;115(5):1385-8. [Medline].

30. Mortimer PS, Dawber RP, Gales MA. A double-blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol. Sep 1986;115(3):263-8. [Medline].

31. Moschella SL. Hidradenitis suppurativa. Complications resulting in death. JAMA. Oct 3 1966;198(1):201-3. [Medline].

32. Moul DK, Korman NJ. The cutting edge. Severe hidradenitis suppurativa treated with adalimumab. Arch Dermatol. Sep 2006;142(9):1110-2. [Medline].

33. Mustafa EB, Ali SD, Kurtz LH. Hidradenitis suppurativa: review of the literature and management of the axillary lesion. J Natl Med Assoc. Mar 1980;72(3):237-43. [Medline].

34. O'Loughlin S, Woods R, Kirke PN, Shanahan F, Byrne A, Drury MI. Hidradenitis suppurativa. Glucose tolerance, clinical, microbiologic, and immunologic features and HLA frequencies in 27 patients. Arch Dermatol. Jul 1988;124(7):1043-6. [Medline].

35. Ramasastry SS, Conklin WT, Granick MS. Surgical management of massive perianal hidradenitis suppurativa. Ann Plast Surg. Sep 1985;15(3):218-23. [Medline].

36. Ritz JP, Runkel N, Haier J, Buhr HJ. Extent of surgery and recurrence rate of hidradenitis suppurativa. Int J Colorectal Dis. 1998;13(4):164-8. [Medline].

37. 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].

38. Rose RF, Goodfield MJ, Clark SM. Treatment of recalcitrant hidradenitis suppurativa with oral ciclosporin. Clin Exp Dermatol. Jan 2006;31(1):154-5. [Medline].

39. Stellon AJ, Wakeling M. Hidradenitis suppurativa associated with use of oral contraceptives. BMJ. Jan 7 1989;298(6665):28-9. [Medline].

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

41. Tennant F Jr, Bergeron JR, Stone OJ, Mullins JF. Anemia associated with hidradenitis suppurativa. Arch Dermatol. Aug 1968;98(2):138-40. [Medline].

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

43. Venturini R, Ruggieri V, Stellar A. The after-effect of movement induced with a rotating spiral (spiral after-effect): reliability and correlation with personality characteristics. Boll Soc Ital Biol Sper. Feb 15 1974;50(3):118-24. [Medline].

44. Von Der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol. May 2000;142(5):947-53. [Medline].

Keywords

Verneuil disease, acne inversa, apocrinitis

Contributor Information and Disclosures

Author

Naveen Pokala, MBBS, MS, FRCS, Staff Physician, Department of Surgery, Bronx Lebanon Hospital
Disclosure: Nothing to disclose.

Coauthor(s)

Ravi Pokala Kiran, MBBS, MS, FRCS (Eng), FRCS (Glas), Staff Physician, Department of General Surgery, St Mary's Hospital
Disclosure: Nothing to disclose.

Suhasini Yadlapati, MBBS, Staff Physician, Department of Surgery, Gandhi General Hospital, India
Disclosure: Nothing to disclose.

Ajay K Shah, MD, Consulting Surgeon, Department of Surgery, Bronx-Lebanon Hospital Center Healthcare System
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

David L Morris, MD, PhD, Professor, Department of Surgery, St George Hospital, University of New South Wales, Australia
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other; AstraZeneca Grant/research funds Other

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)