Continuing medical education
Hidradenitis suppurativa: A comprehensive review

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Hidradenitis suppurativa, also known as acne inversa, is a chronic, often debilitating disease primarily affecting the axillae, perineum, and inframammary regions. Prevalence rates of up to 4% have been estimated. Our understanding of the disease has changed over time. It is now considered a disease of follicular occlusion rather than an inflammatory or infectious process of the apocrine glands. Clinically, the disease often presents with tender subcutaneous nodules beginning around puberty. The nodules may spontaneously rupture or coalesce, forming painful, deep dermal abscesses. Eventually, fibrosis and the formation of extensive sinus tracts may result. The location of the lesions may lead to social embarrassment and the failure to seek medical treatment. Therapies in the past have consisted of long-term antibiotics, antiandrogens, and surgery. New treatments like tumor necrosis factor–alfa inhibitors have given clinicians more options against this difficult disease.

Learning objectives

After completing this learning activity, participants should be able to describe the clinical presentation, demographics, and prevalence of hidradenitis suppurativa, be familiar with current controversies regarding the pathogenesis of this complex, and be able to discuss potential treatments and their outcomes.

Section snippets

Demographics

Key points

  1. HS tends to begin in the second or third decades of life

  2. Females are more likely to develop HS

  3. Recent studies suggest a prevalence of about 1%

HS typically occurs after puberty, with average age of onset in the second or third decades of life. Onset after menopause is rare.14, 15 Isolated case reports have described HS in prepubertal subjects, but these are associated with premature adrenarche.16, 17, 18 HS prevalence may diminish over time. A recent study showed that prevalence among those 55

Clinical presentation

Key points

  1. HS most typically occurs in the axillary, inguinal, perianal, perineal, mammary, and inframammary regions

  2. The distribution pattern corresponds with the “milk line” distribution of apocrine-related mammary tissue in mammals

  3. The most commonly affected site is the axilla

  4. Perianal HS is associated with more debilitating outcomes

HS is characterized initially by the presence of tender subcutaneous nodules. With time, the nodules may rupture, resulting in painful, deep dermal abcesses.1 The lesions may

Clinical staging

Key points

  1. Hurley's staging system is simple and relies on the subjective extent of diseased tissue

  2. The Sartorius system is more sophisticated then Hurley's and is likely to supplant it as a means for conducting clinical trials

One of the first staging systems for HS was proposed by Hurley (Table I).36 Hurley separated patients into three groups based largely on the presence and extent of cicatrization and sinuses (Fig 2, Fig 3, Fig 4).36 It has been used as a basis for clinical trials in the past and is a

Differential diagnosis

Key points

  1. The differential diagnosis for HS is extensive, but can easily be differentiated from other diseases by the age of onset and characteristic locations of the lesions

The differential diagnosis for HS is extensive (Table II) but is usually easy to discern from other diseases.3, 39 HS can be differentiated from other diseases by the appearance of the lesions, postpubertal age of onset, characteristic locations, resistance to antibiotics, recovery of multiple species (rather than a single species)

Etiopathogenesis

Key points

  1. Long thought to be a disorder of apocrine origin, it is now thought to be caused by follicular occlusion

  2. Families with autosomal dominance type inheritance have been reported

  3. Hyperandrogenism likely does not play a role in HS

  4. Bacteria are probably secondary colonizers, which may exacerbate HS, but they are not the primary etiologic agents

  5. Poor hygiene does not cause HS

  6. Some investigators postulate that there may be an underlying immunologic aberration involved in the pathogenesis of HS; more work

Psychological and physiologic morbidity

Key points

  1. HS patients experience a quality of life worse than those with alopecia, mild to moderate psoriasis, and several other dermatologic conditions

  2. HS results in economic and psychological disability in addition to the physical problems

von der Werth et al84 measured morbidity in 114 HS patients using the DLQI questionnaire and demonstrated a total mean score of 8.9, which ranks above alopecia, mild to moderate psoriasis, and several other dermatologic conditions. This high score was heavily

Disease associations

Key point

  1. HS is associated with other diseases of the follicular occlusion tetrad, acne vulgaris, KID syndrome, IK, DDD, SAPHO syndrome, CD, Jackson–Lawler type pachyonychia congenital, reflex sympathetic dystrophy, and FFD, among others

HS has been associated with many disorders (Table III). The other disorders of follicular occlusion (acne conglobata, dissecting cellulitis of the scalp, and pilonidal cyst) are well known associations with HS, and make up the so-called follicular occlusion tetrad.86, 87

Prognosis and disease complications

Key points

  1. The risk of SCC and other malignancies may be increased in those with HS

  2. Contractures, strictures, serious infection, and anemia are also potential sequelae

HS can cause significant complications (Table IV). There have been numerous reports that have associated HS with SCCs of the skin.24, 86, 101, 128, 129, 130, 131, 132, 133, 134, 135, 136, 137, 138, 139, 140, 141, 142, 143, 144, 145, 146, 147, 148, 149, 150, 151, 152, 153, 154, 155 The ratio of SCC in HS favors males (4:1).128 Sixty-one

Treatment

Key points

  1. There is no uniformly effective single therapy for HS; therefore, clinicians will likely have to try an array of different treatment modalities depending on the patient's disease

  2. For patients with extensive disease, wide excision can dramatically improve the patients' quality of life

  3. Most of the listed therapies are ones that dermatologists possess intimate knowledge of and are thus in the best position to treat this debilitating disorder.

Approach to treatment

By this point, readers have likely come to understand the limited nature of clinical treatment trials that have been performed on patients with HS. There is no overwhelmingly effective treatment for patients with any stage of disease with HS. Therefore, most physicians will be forced to try a variety of different therapeutic modalities. We suggest a treatment algorithm based upon the Hurley classification or a tiered approach (Table V). Those with more severe disease may benefit from more

Conclusion

The prevalence of HS may equal or even surpass that of psoriasis, yet it receives only a fraction of the attention and scholarly contributions. Though no overwhelmingly effective treatments have been described, novel therapies are emerging. Many—if not all—of these therapies are well known to dermatologists; therefore, we should play a lead role in managing this debilitating disorder. As our understanding of this disorder grows, so will our treatment options. Readers may find the book by Jemec

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    Conflicts of interest: None declared.

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