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
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Table
| Keratin comedones ¯ Occlusion of the apocrine ducts ¯ Superimposed inflammation and infection ¯ Abscess formation ¯ Chronic infection and spread ¯ Induration and sinus and fistula formation |
| Keratin comedones ¯ Occlusion of the apocrine ducts ¯ Superimposed inflammation and infection ¯ Abscess formation ¯ Chronic infection and spread ¯ Induration and sinus and fistula formation |
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.
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| References |
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References
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Further Reading
Keywords
Verneuil disease, acne inversa, apocrinitis
