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Hidradenitis Suppurativa
Gregor B.E. Jemec, M.D., D.M.Sc.
This Journal feature begins with a case vignette highlighting a common clinical problem.
Evidence supporting various strategies is then presented, followed by a review of formal guidelines,
when they exist. The article ends with the author’s clinical recommendations.
A 36-year-old woman has recurrent boils under both arms and in the groin. They
flare premenstrually, causing pain, suppuration, and an offensive odor. Scarring has
developed in the groin area, and chronically draining sinus tracts are interspersed
with normal skin. Treatment with short courses of antibiotics or with incision and
drainage has had no apparent effect, and she has become socially isolated because of
embarrassment about her condition. How would you manage this case?
From the Department of Dermatology, Hidradenitis suppurativa1,2 is a chronic, recurrent inflammatory disease affecting
Roskilde Hospital, Roskilde, Denmark; skin that bears apocrine glands. It usually develops after puberty, manifested as pain-
and Faculty of Health Sciences, Universi-
ty of Copenhagen, Copenhagen. Address ful, deep-seated, inflamed lesions, including nodules, sinus tracts, and abscesses.
reprint requests to Dr. Jemec at the De- In most patients, flares are accompanied by increased pain and suppuration at vary-
partment of Dermatology, Roskilde Hos- ing intervals, often occurring premenstrually in women. If untreated, the flares sub-
pital, Roskilde DK-4000, Denmark, or at
gbj@regionsjaelland.dk. side within 7 to 10 days.3
European studies have suggested that hidradenitis suppurativa is not a rare dis-
N Engl J Med 2012;366:158-64. ease. A French community-based study,4 in which persons older than 15 years of age
Copyright © 2012 Massachusetts Medical Society.
responded to a validated questionnaire (with a positive predictive value of 85 to
89%), showed a point prevalence at 1 year of 1%. Studies of young adults (18 to 33
years of age) undergoing screening for sexually transmitted diseases have shown
point prevalences of up to 4%.5
Women are more frequently affected than men (female:male ratio, 3:1) and ap-
pear to be more likely to have genitofemoral lesions. The condition most commonly
An audio version
of this article
develops in persons in their early 20s, although the onset has been described in
is available at prepubertal children and in postmenopausal women as well.4,6 The prevalence of
NEJM.org the disease appears to decline at an age of more than 50 years.4
About one third of patients with hidradenitis suppurativa report a family his-
tory of the disease, and affected families with an autosomal dominant mode of
inheritance have been identified. In a small number of cases in which hidradenitis
suppurativa is accompanied by severe acne and perifolliculitis capitis, the disease
has been linked to chromosome 1p21.1–1q25.3 and mutations of the γ-secretase
complex.7 Studies have not shown associations between HLA antigens and hidrad-
enitis suppurativa.8
Cigarette smoking is a recognized risk factor for both the development of hi-
dradenitis suppurativa and severe disease.9 Obesity is also a risk factor; the major-
ity of patients are overweight, and both body-mass index and tobacco smoking have
been directly correlated with the severity of this condition.10
The disease has a substantial negative effect on the quality of life of affected
persons, as compared with the general population or with patients who have other
chronic skin conditions (e.g., psoriasis or eczematous dermatitis).11-13 Rates of sick
HIDRADENITIS SUPPURATIVA
• Hidradenitis suppurativa is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands.
• It is manifested as painful, deep-seated, inflamed lesions, including nodules, sinus tracts, and abscesses, and is esti-
mated to affect 1% of the population.
• A long delay in diagnosis is common, since the disease is often mistaken for a simple infection.
• Lesions treated with incision and drainage routinely recur.
• Few randomized trials have been carried out to guide care, but observational data suggest that many patients have im-
provement after treatment with antibiotics; a tetracycline or a combination of clindamycin and rifampin is often used.
• For more severe disease, treatment may involve immunosuppressive agents such as inhibitors of tumor necrosis
factor α, although results of randomized trials have been inconsistent.
• For recalcitrant lesions, complete removal by surgical excision or laser therapy often results in local clearing.
leave from work are higher and self-reported lation and usually involves the peripheral joints,
general health is lower among patients with often in an asymmetric manner.1
hidradenitis suppurativa than in the general popu-
lation.14 Patho gene sis
Conditions reported to be associated with hi-
dradenitis suppurativa include severe acne, acne The pathogenesis of hidradenitis suppurativa re-
conglobata, and pilonidal cysts, although it is pos- mains unclear. Histologic studies suggest that it
sible that these conditions are misdiagnosed in is a multifocal disease, in which atrophy of the
patients with hidradenitis suppurativa.1 Data from sebaceous glands is followed by an early lympho-
an epidemiologic study suggested a 50% increase cytic inflammation and hyperkeratosis of the
in the risk of cancer of any kind in patients with pilosebaceous unit and, later, by hair-follicle de-
hidradenitis suppurativa, as compared with the struction and granuloma formation.17-22 It is spec-
general population15; specific cancers reported ulated that subsequent healing processes (not well
to occur more frequently in these patients included defined) produce scarring and sinus tract forma-
squamous-cell carcinoma (e.g., Marjolin’s ulcers tion — processes that are exacerbated by the im-
associated with chronic lesions of hidradenitis paired mechanical integrity of the sinus tract epi-
suppurativa, primarily of the buttocks), buccal can- thelium.22 Recent investigations suggest that the
cer, and hepatocellular cancer. However, this study interleukin-12–interleukin-23 pathway and tumor
did not adjust for status with respect to cigarette necrosis factor α (TNF-α) are involved in the patho-
smoking, and it is likely that the observed asso- genesis of hidradenitis suppurativa, supporting the
ciations were confounded by smoking — a rec- proposition that it is an immune or inflamma-
ognized risk factor for both hidradenitis suppura- tory disorder.23,24
tiva and these diseases.
The frequency of hidradenitis suppurativa has S t r ategie s a nd E v idence
been reported to be increased among patients
with Crohn’s disease, affecting 17% of such pa- Evaluation
tients, according to one report. A relationship be- The diagnosis of hidradenitis suppurativa is gen-
tween the two conditions is supported by clini- erally made clinically. On physical examination,
cal, histologic, and epidemiologic similarities, one may see characteristic inflamed and nonin-
such as sinus tracts, granulomatous inflamma- flamed nodules; draining and nondraining sinus
tion, scarring, and onset after puberty.16 Arthritis tracts; and abscesses in the axillary, inguinal, and
(rheumatoid factor–negative and HLA-B27–nega- anogenital regions. The lesions occasionally ex-
tive) is also more frequent among patients with tend beyond these areas and appear around the
hidradenitis suppurativa than in the general popu- anus, on the buttock, or on the breast in females.
A B C
The nodules are located in the deeper dermis and Assessment of the severity of the disease is
are rounded rather than having the pointed, pu- helpful in guiding treatment and is generally based
rulent appearance of simple boils (Fig. 1, 2, and 3). on the Hurley staging system (Fig. 1).29 In the ma-
Secondary lesions such as pyogenic granulomas jority of cases, patients have stage II disease at the
in sinus tract openings, plaquelike induration, time of diagnosis, presumably reflecting diagnos-
ropelike scars, and giant multiheaded comedones tic delay. Only about 1% of patients have progres-
may also be found. sion to stage III disease.
In selected cases, additional testing may be
helpful. Biopsies and bacterial cultures are indi- Medical Management and Lifestyle Measures
cated only in atypical or refractory cases. Routine Stage I (localized) disease is usually managed with
bacteriologic studies of the lesions in hidradeni- topical therapy, whereas systemic therapy is rec-
tis suppurativa are most frequently negative, al- ommended for more widespread or severe disease.
though flares may be associated with superin- Since data from randomized trials are limited,
fection involving a range of bacteria, including choices among treatments are generally guided
Staphylococcus aureus.25,26 If extensive surgery is by the results in case series and by clinical expe-
planned, ultrasonography may help in the pre- rience.
operative assessment by identifying subclinical In a small, randomized, placebo-controlled trial
extension of the lesions.27 involving patients who appeared by description to
Despite the typical presentation, the disease is have mild disease (although no formal staging was
often diagnosed only after a considerable delay; performed), topical administration of clindamy-
in one study, the median delay was 12 years.28 cin (10 mg per milliliter twice daily) was found to
Many cases are misdiagnosed as common boils reduce the number of abscesses, nodules, and pus-
and treated with lancing or short-term antibiotic tules at monthly evaluations during a 3-month
therapy — approaches that may seem effective at course of treatment.30 Clinical experience has also
first (since flares tend to subside spontaneously supported the use of intralesional injections of
after a week) but ultimately fail. glucocorticoids (e.g., triamcinolone, 2 to 5 mg)
ment in severe cases. Similarly, there is a need for cinolone, clinical experience suggests that it may
a systematic comparison of surgical techniques be useful for some isolated lesions. Case series
(e.g., laser vs. conventional surgery) and approach- suggest that scarred lesions are best treated with
es to postprocedure management (open healing wide excision or evaporation with the use of a
vs. primary closure or skin grafting). carbon dioxide laser. More extensive and severe
disease requires systemic treatment. For stage II
Guidel ine s disease, as seen in the patient described in the
vignette, I would try combination antibiotic ther-
No formal guidelines are currently available for the apy (clindamycin and rifampin, 300 mg twice daily
management of hidradenitis suppurativa. for 6 months), since it has appeared to be effec-
tive in case series and clinical practice, although
the combined regimen has not been compared
C onclusions a nd
R ec om mendat ions with either of these agents alone or with other
treatments in randomized clinical trials.
The patient described in the vignette presented Dr. Jemec reports receiving consulting fees from Abbott Lab-
with a history of recurrent lesions that are consis- oratories, Merck, Pfizer, and Dumex-Alpharma, lecture fees from
tent with stage II hidradenitis suppurativa. Man- Galderma and Pfizer, and grant support from Abbott Laborato-
ries, Photocure, and LEO Pharma; receiving equipment on loan
agement should be guided by disease severity. from Michelson Diagnostics; and receiving reimbursement for
For stage I, characterized by mild, nonscarring travel expenses from Abbott Laboratories, Galderma, and Photo-
disease, limited clinical trial data support the ef- cure. No other potential conflict of interest relevant to this article
was reported.
ficacy of topical clindamycin; although data are Disclosure forms provided by the author are available with the
lacking to support the use of intralesional triam- full text of this article at NEJM.org.
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