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DOI: 10.1111/j.1468-3083.2009.03356.x

INVITED ARTICLE
Blackwell Publishing Ltd

Hidradenitis suppurativa
J Revuz*
11 Chausse de la Muette, 75016 Paris, France
*Correspondence: J Revuz. E-mail: revuz.jean@wanadoo.fr

Abstract
Hidradenitis suppurativa is a chronic disease characterized by recurrent, painful, deep-seated, rounded nodules and
abscesses of apocrine gland-bearing skin. Subsequent suppuration, sinus tracts and hypertrophic scarring are its main
features. Onset is usually after puberty, although it is most common during the third decade and may persist in old age.
The disease tends to be chronic and may develop to subcutaneous extension leading to indurations, sinus, and fistula
having a profound impact on the quality of life. The prevalence is 1% in several studies. Axillary and inguinal involvement
is more common in females; peri-anal and buttocks localizations are prevalent in males. The exact aetiology remains
unknown. The primary event is a follicular occlusion with secondary inflammation, infection and destruction of the
pilo-sebaceo-apocrine apparatus and extension to the adjacent sub-cutaneous tissue. Infection is common. Smoking
may be a triggering factor. Obesity aggravates the discomfort. Differential diagnostic includes Crohns disease, nodular
acne and furonculosis. The main complications are arthropathy, carcinoma. Treatment depends upon the stage of the
disease. Early nodular lesions may be treated by antibiotics for acute stage; long-term antibiotics, zinc salts may be useful
as maintenance treatment; anti-TNF drugs have been used in severe cases; systemic steroids, estrogens, antiandrogens, retinoids have been used as options with limited success. Surgical treatment includes incision with or without
drainage for limited abscesses; limited excisions are used for locally recurring draining sinuses. Total wide excision and
healing with secondary intention or flaps and grafts is the only curative procedure in case of advanced disease.
Received: 27 March 2009; Accepted: 25 May 2009

Keywords
acne inversa, follicular diseases, hidradenitis suppurativa, treatment

Conflicts of interest
None declared.

Historical background
Hidradenitis suppurativa (HS) was first described during the
year 1854 by a French surgeon Aristide Verneuil; the original
paper was Etudes sur les tumeurs de la peau; de quelques
maladies des glandes sudoripares.1 The word tumeur at that
time did not mean tumour but enlargement. From the beginning,
HS was linked to the sweat glands but in fact apocrine sweat
glands were only described in 1921. From the beginning, debates
took place about the precise location of the initial phenomenon;
in 1955, Shelley 2 after an experiment of occluding the saxillary
skin concluded that the primary event was a keratinous
plugging of the apocrine sweat duct. Later, Yu3 showed that
follicular occlusion was the most constant feature of HS and
probably the primary event. Kligman and Plewig4 had already
considered that the follicular occlusion was the main event and

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drew this conclusion by grouping together HS, acne conglobata,


dissecting folliculitis of the scalp and later pilonidal cyst,
naming this association follicular tetrad and renaming HS to
acne inversa.

Definition
Hidradenitis suppurativa (HS) is defined only by its clinical
features and its chronicity; there is no biological or pathological
test to help. It is recognized by the presence of recurrent, painful,
deep-seated, rounded nodules usually ending in abscesses and
sinus tracts with suppuration and hypertrophic scarring of apocrine
gland bearing skin.
A consensus definition was adopted by the second congress
organized by the Hidradenitis Suppurativa Foundation (San
Francisco March 2009):5

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HS is a chronic, inflammatory, recurrent, debilitating, skin follicular


disease that usually presents after puberty with painful deep seated,
inflamed lesions in the apocrine gland-bearing area of the body,
most commonly, the axillary, inguinal and anogenital regions.

Epidemiology

1 Typical lesions, i.e. deep-seated painful nodules: blind boils


in early lesions; abscesses, draining sinus, bridged scars and
tombstone open comedos in secondary lesions.
2 Typical topography, i.e. axillae, groin, perineal and perianal
region, buttocks, infra and inter mammary folds.
3 Chronicity and recurrences.
These three criteria must be met for establishing the diagnostic.

Prevalence

Despite the reputation of HS as a rare disease, a global prevalence


of 1% in the general population has been reported in several
studies reviewed by Naldi.6 A recent study performed by a poll
institute has confirmed this very high prevalence.7 The discordance
with the apparent rarity in clinical experience could be explained
by the prevalence of mild forms, the weariness of patients
discouraged by the results of treatments and by the poor
knowledge of HS in the medical community, which is revealed by
the diagnostic delay (usually several years).
A peak prevalence of 4% of self-reported cases in a population
of young female adults in Denmark was recorded and was likely
due to a high prevalence in young adults.8 This was objectively
confirmed by examination of patients in another study9 of the
same team. Although not rare, HS is an orphan disease due to the
lack of HS knowledge in the medical community and to the poor
results of most therapeutic interventions.
The sex ratio is 1/3.3.
Risk factors

Risk factors have been reviewed by Naldi.6 A recent case control


study of factors associated with HS in 302 patients7 has confirmed
that smoking and overweight are the two main factors associated
with HS. Smoking was significantly more common (more than 70%)
in HS patients than in controls: a strong association in multivariate
analysis with current smoking was demonstrated [OR = 12.55,
(8.5818.38)]. No temporal relationship, no dose effect has been
demonstrated; thus, smoking while highly associated with HS
cannot be considered as a true risk factor. Furthermore, no
specific mechanism to explain this association has been provided
and the effect of smoking cessation is not documented. Association
with overweight and obesity was observed in the same study with
a high risk: [OR = 1.12, (1.081.15) for each increase of one unit
of body mass index]; as there is dose/effect relationship, overweight
and obesity may be true risk factor. Yet a clear demonstration
would rely on a temporal relationship and an effect of reducing
weight on the evolution capacity of the disease.
Among non-genetic factors, uses of antiperspirants, talc and
deodorants, hair removal by a safety razor have been ruled out.10
Tight clothing may contribute to discomfort. Genetic and hormonal factors are considered below.
Diagnostic criteria (adopted by the Congress of the HSF
Foundation March 2009)5

The diagnostic relies on the presence of:

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Clinical description11
The onset of disease is usually after puberty, usually during the
second and third decades. There is frequently a long delay before
the diagnostic is established, particularly in mild intermittent cases:
a nodule or an abscess is surgically treated by incision and packing
and the chronic and relapsing nature of the disease is not considered.
Moreover, there is a paucity of knowledge of this disease among
general practitioners, surgeons and even dermatologists.
Primary lesions

The early lesions are solitary, painful nodules that may persist for
weeks or months without any change or with occasional episodes
of inflammation (Fig. 1). Early isolated lesions are not characteristic
and frequently considered as boils or common abscesses. This
may explain why the diagnostic is frequently delayed. The shape
of these nodules, round rather than pointed, without central necrosis,
and their topography should help to differentiate them from
common boils and lead to a correct diagnostic. The deep situation
in the hypodermis makes them sometimes barely visible (Fig. 3).
They are extremely painful. Approximately 50% of all patients
experience subjective prodromal symptoms such as burning, stinging,
pain, pruritus, warmth and/or hyperhidrosis, 12 to 48 h before
overt nodule occurs. Mean duration of a single painful nodule is
715 days. The nodules may remain blind, i.e. fail to burst and
may resolve spontaneously or persist as a silent nodule with

Figure 1 Axillary nodular superficial lesion with an abscess


formation.

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987

Figure 2 Sinus tract and pyogenic granulomas.

Figure 4 Hypertrophic rope-like scar.

Figure 3 Deep-seated nodular lesion.


Figure 5 Open comedos in burn out lesions.

inflammatory recurrences. The most frequent evolution, at least


in severe cases, is the evolution toward abscesses (Fig. 2), and an
external rupture, spontaneously or after incision, draining
purulent material. Rupture of abscesses into the neighbouring
structures is exceptional.
Secondary lesions

The repetition of attacks may lead to chronic sinus formation, with


intermittent release of serous, purulent, or bloodstained discharge,
frequently foul odours from anaerobic colonization. It persists for

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months, sometimes years, unless properly excised. Ulcerations,


pyogenic granulomas (Fig. 2), sometimes occur. A characteristic
secondary lesion is constituted by hypertrophic scars: healing
occurs with dense fibrosis, which may appear as indurate plaques,
or as linear rope-like bands (Fig. 4). Multiporous or uniporous
tombstone open comedos are frequently observed usually as
tertiary lesions in burnout regions (Fig. 5). Closed comedos are
not observed within HS lesions.

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Figure 6 Locations prevalence in both


sexes; the statistical differences between
men and women are shown ( data from 302
patients; ref 11).

There is no regional lymphadenopathy. Pathological specimens


are usually not taken from patients with HS as histopathology is
not helpful for diagnostic and/or therapeutic purposes.

requires excision (frequently already excised when HS appears) or


as a little midline developmental depression of the intergluteal
furrow.12

Associated lesions

Clinical course and severity grading

Follicular papules and pustules are frequent in area of HS as well as


in other areas; they do not constitute a diagnostic clue for HS. On
the buttocks, folliculitis can leave round, slightly depressed scars.
Epidermal cysts are prominent in some patients: round smooth
elastic nodules, 16 cm in diameter, located on external genital
organs, on the face or on the thorax.

Chronicity is the hallmark of HS. The mean age at the beginning


of the disease is 22.1 years ( 8.2); prepubertal cases are exceptional.
For patients with pre-existing acne, the disease frequently starts
after acne has disappeared. The mean duration of the active
diseases was 18.8 years in a questionnaire survey of Von der
Werth.12 The disease begins earlier for patients with family history
of HS. The disease tends to become less active among females in
their 50s and is usually in complete remission after menopause.
Premenstrual flares are commonly reported. Pregnancy and
breast feeding are usually periods of complete or partial remission.
In males, HS may continue to be active in old age13 (Fig. 7).
Severity is roughly defined by the classical, yet still useful,
classification of Hurley14 (Table 1). Most practitioners (specially
the surgeons) only know the most dramatic picture of severe
disease. In fact, a better knowledge shows that the mild forms are
more frequent. In a personal series of 302 patients, the mildest
grade (Hurley I) constitutes 68.2% of the patients, intermediate
severity (Hurley II) 27.6%, and the most severe and dramatic HS
(Hurley III) 3.9%.12 Hurleys grades are helpful to classify a patient
(or rather a localization, i.e. armpit, groin) in a category of severe
or mild involvement. However, it is not really a severity index
helpful for managing patients.
Seriousness and course of the disease are various:
Intermittent benign course. This mild yet chronic and
painful disease (Hurleys stage I) may be relentless and
progressive or with periods of acute exacerbations frequently
as premenstrual flares (33/65 women experienced premenstrual

Topography8,1012

The affected sites are, by order of frequency: groins including


inner thighs, pubic region, scrotum, vulva; armpits; perianal
and perineal regions; intermammary and inframammary folds,
buttocks. These locations are along the milk line of apocrine and
mammary tissue which have the same embryonic origin. Groin
and submammary region are most commonly affected in females;
buttocks and peri-anal skin in males; the anal canal is usually
spared. Figure 6 shows the respective frequency of involvement in
both sexes. Several sites usually symmetric may be simultaneously
affected; recurrences occur in and around the original site. Atypical
locations are sometimes observed: waist, abdomen, specially
peri-umbilical region, and thorax. In retro-auricular fold, there
are frequently epidermal cysts usually non-inflammatory. The nape
of the neck may be involved in a way similar to what is observed
in severe acne and/or in the so-called acne keloidalis nuchae
which would be better called fibrosing folliculitis. These atypical
locations are more frequent in males.12
A pilonidal sinus is extremely frequent (30%, more frequent in
males) either as a true nodular and suppurating lesion which

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Hidradenitis suppurativa, a review

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Figure 7 Age and sex repartition of HS patients at first visit.

Table 1 Hurleys classification


Grade I

Abscess formation, single or multiple without sinus


tracts and cicatrization

Grade II

Recurrent abscesses with tract formation and


cicatrization. Single or multiple, widely separated lesions

Grade III

Diffuse or near-diffuse involvement, or multiple


interconnected tracts and abscesses across entire area

flares according to Jemec8) and remissions; complete


spontaneous remissions of several weeks, or even months are
experienced. These patients are very frequently misdiagnosed
as furonculosis or considered to have recurring abscesses
which are incised in emergency ward without a more precise
diagnostic. Some of these patients are able to predict the
occurrence of a new attack 24 h before due to specific premonitory
symptoms like pain, itching, malaise.
The severity in these cases may be evaluated by the
number, intensity and duration of painful episode per year.
Continuous intermediate course. This may correspond to
Hurley I or II. The disease is continuous; the number of
locations involved, the extent of lesions in each area, the
intensity of pain, number of days per month with pain and
suppuration are the main factors of severity. There is an
extreme variety of clinical pictures. In a defined area, a new
attack may be the re-occurring inflammation of a pre-existing
fistula or the burst of one or several new nodules/abscesses.
The way to manage the patients is not the same in these two
situations.
Severe course. Two different types of severe disease may be
observed:
A permanent involvement of an area, which forms an infiltrated, painful, suppurating mass with coalescent nodules,
fistulas, abscesses, draining sinuses and fibrous scars
(Fig. 8). No normal skin exists between the lesions. The


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Figure 8 The terrible Hurley grade III.

patient experiences pain, discomfort, swelling, malodorous


discharge, limitation of motility. It is the typical Hurleys
grade III. Radical surgery is the only reasonable treatment.
Alternatively the patient may have a multitude of nodules
and abscesses separated by normal skin; each lesion has
duration of 10 to 30 days but with overlapping evolution
and new lesions appear on place where there was no previous
lesion. In this case surgery is unhelpful and medical treatment should be given.
When assessing the global severity of a patient, one has to
take into account the facts that:
The severity of involvement is not parallel between various
locations: groin may be in grade III and armpit free of disease.
Evolution from one grade to another, e.g. from I to II or III is
seldom observed: patients with mild disease remain mild and
most severe cases seem to be severe since the start.


Quality of life impairment


A profound alteration of the quality of life is observed even in
mild cases. A study of 114 patients with HS using the DLQI index
recorded a very high score particularly for question 1 measuring

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Figure 9 Lymphedema of the pubis : a complication of HS.

the level of pain, itching and soreness.15 A recent study of 61


patients with HS using several quality of life questionnaires
showed that the impairment of quality of life in each dimension
was statistically higher (P < 0.001) than for neurofibromatosis,
urticaria, psoriasis and atopic dermatitis.16 This was particularly
dramatic for self-perception, daily living activities, mood state,
social functioning and physical discomfort. Time spent, delay in
diagnostic, loss of work was observed in a series of patients by
Jemec et al.17

Figure 10 Squamous cell carcinoma on HS: always on the


buttocks in men; bad prognostic.

Associated diseases

histological picture is considered by many pathologists as the


initial event of HS. Early biopsy of HS in normal-looking skin,
however, has not been performed; hence, this early follicular
occlusion may in fact be a secondary phenomenon. The
controversy is open. Association of HS with severe nodular acne
(acne conglobata) and/or with dissecting cellulitis of the scalp and
pilonidal cysts has been reported. Pilonidal cyst is frequently
associated with HS: 30% if both true sinus and midline intergluteal
dimple are taken into account. 12 Moreover, comedones are
frequent in both acne and HS. According to these findings, some
authors have proposed to rename HS acne inversa and to include
the above-mentioned diseases in an acne tetrad.4 In fact,
concomitant acne is not frequent in HS (20% in males, 10% in
females); a past history of significant acne (long lasting, leaving
scars) is recorded in 44% of males 23% of females;12 Dissecting
folliculitis of the scalp is exceedingly rare (1%).12 Association of
the four components of the so-called acne tetrad was never
encountered in 500 patients (personal observation). A prospective
study of 70 females with HS and 100 controls failed to find any
difference in the prevalence of acne, hirsutism or irregular periods
between the two groups.8 Contrary to the initial lesions of acne,
comedos in HS are never closed; they are a secondary tombstone
comedo frequently double-ended open comedos, i.e. blackheads.
Elevated sebum excretion, which is a major pathophysiological
feature of acne, is absent in HS.21 Moreover, effective treatments
of acne, isotretinon and anti-androgens, are not effective in HS
(see below). Hence, if hidradenitis suppurativa is not a good
denomination of the disease, acne inversa is not better and
should be discarded until a better knowledge of the pathophysiology
may help to find a better name.

Acne and follicular tetrad

Dowling Degos & Kitamuras disease

Histopathological studies demonstrate that a striking feature of


HS is a follicular occlusion from unknown mechanisms. This

Reticulate pigmentation of the flexures may be sometimes


associated with HS. It is difficult to say if this is a significant

Complications
Acute infectious complications such as cellulitis or systemic
infection are very unusual.
Lymphatic obstruction and lymphedema,18 (Fig. 9) scrotal
elephantiasis may complicate long-standing inflammation.
Squamous cell carcinoma may arise (Fig. 10). It is quasi
exclusively observed in men in buttock localizations after
several decades of evolution. It often starts in deep situation;
thus, the diagnostic is tardy and the prognostic usually very
bad.19 An epidemiological survey found an elevated incidence
of skin and visceral cancers in HS.20
The complications of long-standing untreated disease: fistulae
formation into the urethra, bladder, rectum, or peritoneum as
well as the consequences of chronic suppuration such as anaemia, hypoproteinemia, amyloidosis are historical complications which have become exceptional today. Peripheral or
axial arthropathy, a classical complication, and SAPHO syndrome, are not frequently encountered.
Chronic malaise and depression are frequent.

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Hidradenitis suppurativa, a review

association as usually people do not seek advice for these


pigmentary changes which are discovered during examination of
groin and armpits of HS patients.22
Crohns disease23

It is both a differential diagnostic and an associated disease.


Cutaneous Crohns disease must be differentiated from HS in its
peri-anal location and the clinician should always be aware of the
possibility of Crohns disease mimicking HS in case of peri-anal
lesions and eventually perform the necessary investigations.
Atypical condyloma-like lesions seen in Crohns disease are not
observed in HS; conversely, specific Crohns lesions outside the
peri-anal regions are unusual, but it is impossible to differentiate
them on clinical grounds only. The presence of epitheliod
granulomas in the dermis away from the site of active inflammation
may alert, but biopsies are not frequently performed in patients
with HS. Whether a doubt persists, a colonoscopy has to be
performed even in the absence of overt digestive symptoms. True
association does exist including cases of axillary HS. Several case
reports and few series of this association have been reported.24 The
efficacy of anti-TNF drugs in HS was discovered serendipitously
because of this association. Both diseases may be the result of
abnormal hostmicrobial interactions involving pattern recognition
receptors such as toll-like receptors and NODs.25

991

non-infundibular keratinized and non-keratinized epithelium of HS


lesions has been shown to be similar to that observed in the outer
root sheath suggesting its participation in the pathogenesis of HS.30
An echographic study of hair follicles of healthy skin of HS
patients highlighted their wide diameter and distorted shape and
their deep location.31 Magnetic resonance imaging has been used
in some case; it may help to differentiate HS from Crohns disease
or from subcutaneous infection and may be helpful prior to large
excisional surgery.32
Genetic factors33,34

A family history is given by 30% to 40% of patients,12 This figure


may be an underestimation as HS is usually concealed by a
sufferer even to close relatives. An autosomal dominant pattern
has been reported and a linkage to a locus at chromosome 1p21.1
1q25.335 has been reported in a family but with no linkage to other
families. HS appears to be a genetically heterogeneous disease
with several mutations at various locations. No association was
found with CARD15 which is linked to Crohns disease.36 Specific
genetic studies yielded to conflicting results. HLA association is
not significant.37 Patients with a family history have usually a
milder disease with an earlier onset; this raises the question of
heterogeneity of HS. A prerequisite to good genetic studies in the
future relies on the improvement of clinical description and
definition of clearly defined subsets of patients.

Differential diagnosis
Common abscess, carbuncles and furunculosis (recurring common
boils) are the main sources of diagnostic failure of recognizing
early lesions. Infected Bartholins gland, infected or inflamed
epidermal cysts (improperly called steatocystoma multiplex),
lymphogranuloma venereum, scrofuloderma, Actinomycosis,
developmental fistulae, nodular acne and pilonidal cyst, Crohns
disease are classical sources of error. In fact, HS is ignored, not
mistaken for another disease; as soon as the physician thinks of it,
the diagnostic is usually obvious.

Aetiology
The causes of HS are unknown; numerous hypothesis concerning
tobacco, infection, genetics, immunity have been raised and
discussed.26

Infection38

Traditionally, bacteria have been implicated in HS and they play


an important role in most overt clinical manifestations but they
are not the initial causative factor. Furthermore, the absence of
serious infectious complications, i.e. cellulitis, the absence of
abnormal lymph nodes,39 rules out a major direct role for bacterial
infection. A large variety of bacteria can be found: streptococci,
staphylococci, and Escherichia coli in the early stages. But
superficial sampling is frequently sterile or finds bacteria from the
normal flora. Deep sampling is difficult and may be contaminated
by superficial flora. Thus, it is not possible to rely on culture of
lesions to adapt antibiotic treatment. During the chronic relapsing
stages, anaerobic bacteria and Proteus species are commonly present.
This deep flora may be responsible for the destructive and
extensive process which leads to the dramatic Hurleys III features.

Pathology and gross morphology

HS was initially described as a disease of the apocrine gland;


histologic studies reveal that the earliest lesion observed in lesions
excised was a follicular event with infundibular keratinization,
leading to a histological image compatible with occlusion;
inflammation (early lymphocytic infiltration then chronic
inflammatory infiltrate containing histiocytes and giant cells
related to keratin fragments) and necrosis of the sebaceous and/or
sweat gland are secondary phenomenons.27 However, Jemec et al.
recorded that a further histopathological examination reveals a
primary apocrinitis in 5% of all cases28,29 Keratin expression in

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Hormonal factors

Premenstrual flare-ups, female preponderance, frequent occurrence


after menarche, improvement during pregnancy drew the attention
toward hormonal factors and hypothesized hyperandrogenic
syndromes. The usual absence of clinical signs of virilism, the
normality of circulating androgens, the absence of hyperseborrhea,
and the limited effect of anti-androgen treatments, rule out a key
role of hyper androgenism. Signs of virilization are unusual in HS.
Most case series indicate no significant change in serum hormone
levels in HS patients.40 The relationship between HS and

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Table 2 Sartorius score modified by J Revuz


Number

Coefficient

1 Anatomical region involved: armpit, breast, inguino-femoral, perianal and perineal

|__|

x3

Total
|__|__|

2 Lesions: Nodules

|__|

x2

|__|__|

Abcess or fistulas

|__|

x4

|__|__|

Hypertrophic scars

|__|

x1

|__|__|

Others (folliculitis, pustules ... )

|__|

x 0.5

|__|__|

3 The longest distance between two relevant lesions or size if only one lesion.
< 5 cm = 2; < 10 cm = 4; 10 cm = 6; if no active lesions = 0

|__|

x1

|__|

4 Are all lesions clearly separated by normal skin? Yes = 0; No = 6

|__|

x1

TOTAL

hyperandrogenism is largely based on the finding that the free


androgen index is increased because of a low level of sexhormone-binding globulin, which is influenced by body weight.
As body mass index is usually increased in HS, this finding is
common and does not indicate hyperandrogenism. No significant
difference in androgen metabolism was noted in apocrine glands
isolated from HS patients compared with matched controls.41 Some
facts, however, remain unexplained, such as the high prevalence in
females, the frequent improvement during pregnancy, and the
usual termination of the disease in post-menopausal women.
Immunological and other host factors

There is no gross anomaly of the adaptive immune system. Yet the


association with Crohns disease and the successes obtained with
anti-TNF biologics has focused attention on possible alteration
of the innate immune system. Minor alterations of NK cells have
been found.42 An enhanced expression of TLR2 by infiltrating
macrophages and dendritic cells has been shown in the diseased
skin of patients with HS.43 The wide range of possible pathogenic
mechanisms suggested by different studies may imply that HS is
associated with host mechanisms rather than exogenous factors.
Taking into account the paradox that both anti-infectious
(antibiotics) and pro-infectious (anti-TNF, corticosteroids,
immunosuppressive drugs) therapies may be helpful, HS may
appear as an auto-inflammatory disease based on a defect in the
hair follicle innate immunity. Future investigations should target
the Toll receptors, cytokine network and anti-microbial peptides
in the hair follicle and more specifically in the apocrine follicle.23

|__|
|__|__|__|

This classification is useful at least as a guide to choose between


medical or limited surgical treatment (stage I) and large excisional
surgery (stage III). A more precise scoring system i.e Sartorius
score is being used to follow-up patients as an outcome measure46,47
(Table 2). This score has not been formally validated but it has
been useful in several therapeutic studies and has been shown to
be highly correlated to Hurleys classification, intensity and duration
of pain and suppuration which are good markers of inflammation
and of the burden of the disease, as well as the quality of life impairment.12 Pain scale (visual analogue scale or numerical scale) may also
be used as patient-oriented outcome measures. Skin-disease-specific
quality of life questionnaire is also useful: using Skindex-France,
an important impairment of quality of life has been shown13 in
comparison with other skin diseases48 and a very good correlation
with therapeutic results and Sartorius score evolution has been
demonstrated.13 DLQI may also be useful.15 Frequency of flares/attacks
also plays a role in the severity assessment of intermittent cases and
may be analysed as number of flares during a 3-month period.
Therapeutic strategy: medico-surgical management

Treatment depends on the stage (Hurleys classification), frequency


of exacerbation and on the goal of the patient. A permanent cure
can only be obtained by wide surgical excision but such a
procedure is to be considered only in case of advanced disease, i.e.
stage III or severe stage II. Alternatively, early disease may benefit
from medical and medico-surgical approaches.
Medical and surgical treatments are not mutually exclusive. On
the contrary combination, simultaneously or successively, is often
necessary.49

Drugs

HS is not usually related to medications. It has been reported to be


exacerbated by lithium;44 some patients receiving sirolimus after
transplantation have experienced the onset of a de novo HS.45

Treatment
Severity grading

The classical three clinical stages have been defined by Hurley13


(Table 1).

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Acute stage treatment options

Some patients suffer from recurrent painful nodules but also


experience periods of remission. They may benefit from the
following options:
Topical treatments including antiseptics and antibiotics are
advocated by some authors; topical clindamycin has been
claimed to be effective50 and as effective as tetracycline in a
clinical trial.51 As a rule, they are poorly effective due to the
depth of the lesions.

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Journal compilation 2009 European Academy of Dermatology and Venereology

Hidradenitis suppurativa, a review

A short course of antibiotic may be tried for an acute, painful


nodule to shorten the pain duration and to abort it, avoiding
evolution of the lesion toward an abscess. Various antibiotics
have been used for that purpose: amoxicilline, cephalosporine,
clindamycine, rifampicin, M-type penicillin. Amoxicillin +
clavulanic acid may be the more effective regimen provided
that it is taken very early: within 1 h of first signs or of premonitory symptoms. This means that the patient has to carry the
drug every time. A loading dose should be swallowed
immediately (i.e. 3 g for a bodyweight of 70 kg) and followed
by the same dose divided up over the course of the day for the
next few days.
Intralesional steroids (e.g. triamcinolone (510 mg) has been
advocated. Rapid involution (1224 h) of early lesions may be
obtained.52
High doses of systemic steroids may be used to reduce inflammation and pain. They can be used as an alternative to high-dose
antibiotics to abort a lesion, i.e. prevent its evolution toward an
overt abscess or as an association with antibiotics. They have to
be tapered rapidly.53

993

Deep abscesses (an essential characteristic of HS lesions)


are slightly more complicated:
Signs of local inflammation and more general signs
are the same for abscessed and non-abscessed nodules,
although the intensity of the pain is less if not abscessed.
Fluctuation is difficult to evaluate when the nodule is
deep.
The damage caused by inappropriate incisions, particularly in the case of exploratory incisions to find a
non-existent mass, can be significant. Such incisions are
extremely painful, either primarily or secondarily, and
may worsen the lesions.
Thus, a deep, tense abscess that is too painful to bear must be
incised; the decision to pack the wound depends on whether
spontaneous drainage will continue, the risk of premature closure
and the location of the wound. Packing is necessary in case of
very deep cavity, concave area, narrow incision or incomplete
immediate evacuation. Then, it should not be changed too frequently
and should be stopped as soon as it is dry.


Emergency surgical incision

Chronic continuous intermediate course


Drug therapy. Various drugs have been used long-term; the goal is

For an abscess, i.e. when the lesion is fluctuating, full of liquid, the
medical abortive therapy is ineffective and it should not delay the
required surgical drainage (incision) procedure.
Incision of a fluctuating abscess is frequently performed in an
emergency ward. Usually the diagnostic of HS is missed: acute
abscess is the diagnostic. Incision is usually followed by packing;
nursing in the following days and renewal of packing is painful
and usually a nightmare for the patient. A limitation of the
number of incisions and avoiding packing most of the time should
improve the patients quality of life. Some simple statements are to
be written and some simple rules are to be followed:54
Non-abscessing nodules must never be incised.
Fluctuation of a superficial lesion must be established before
incision is contemplated.
The aim of an incision is to evacuate a collection of pus (i.e. an
abscess) in order to alleviate pain.
The goal of this incision is purely an immediate analgesic
effect, pain relief, without affecting the development or course
of the illness, and without full clearance of the lesion incised.
Some abscesses (the more superficial ones) open to the surface
spontaneously; these do not require surgical care as this may
worsen scarring.
Distinguishing between a nodule and an abscess:
Superficial abscesses can be easily recognized by: heat from
the area + whiteness of skin at the centre of the red area +
fever + insomnia or patient awakened in the night by shooting pain. Palpating the area will indicate if it is fluctuant (i.e.
it contains free fluid). An incision is not always required, but
is a simple technique to relieve some of the pain (a desirable
effect). Packing is not necessary in this case.

to stop the evolution, reduce the relapse rate and avoid pain and
chronic suppuration. Due to a probable heterogeneity of the
disease, one particular patient may benefit from a drug which is
useless for others.
Antibiotics. For severely affected patients with a high level of
inflammation, pain and discharge, a 10-week regimen of the
association of clindamycin and rifampicin5557 is very useful.
Patients with Hurleys stage I or II can benefit from this
regimen. A complete remission, sometimes lasting as long as
1 year, is obtained in some patients.
In less severely affected patients or after the 10 weeks of
clindamycinrifampicin treatment, when inflammation and
pain are reduced, a maintenance treatment can be used to prevent or at least alleviate new attacks of nodules and abscesses.
Long-term administration of tetracycline may be helpful. No
data are available on this use of tetracycline despite the fact
that they are widely used.
High doses of zinc salts (90 mg of Zinc gluconate = 3 times
what is used in acne) has proved to be effective in short open
series.58 It helps in maintaining the remission in a significant
number of patients.
Metronidazole particularly in case of bad odour may be helpful.
When surgery is indicated for stage III or severe stage II disease,
a 1-month course of antibiotics prior to surgery is potentially
useful to prevent infectious complications. In addition, this
may better delineate and identify the lesions to be excised, and
effectively downsize the area to be excised.
Antiandrogens. Cyproterone acetate-2 mg/day associated
with estrogens as a contraceptive pill has been compared with
a classical contraceptive pill; no difference was observed

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994

between the two products.59 It may exceptionally be useful at


very high doses, i.e. 100 mg/day in a very limited number of
patients. A retrospective study of 64 females treated with
cyproterone acetate claimed to have demonstrated superiority
upon antibiotic treatment. This result deserves at least a
second look.60 Finasteride has been used in about 10 female
patients with claimed good results.61,62
Retinoids. Isotretinon is ineffective in contrast to what is
observed in acne.63,64 The absence of hyperseborrhea in HS
may explain the difference. Some reports of successful
treatment with etretinate or acitretin have been published
(reviewed65).
Dapsone has been used with good results.66 Such a drug
should be used with caution owing to its very serious side
effects.
Anti-TNF drugs. Several case reports and short series have
claimed a dramatic efficacy of Infliximab,67 Etanercept68,69 and
Adalimumab 70 in severely affected patients. Long-term
efficacy has been claimed for both Infliximab71,72 and Etanercept.73 Beyond the initial enthusiasm for new magic pills, the
real risk/benefit ratio remains to be determined as the usual
bias toward positive publication is clearly at work, and that
conflicting results have been published: only transient efficacy
was observed with Infliximab in a series of seven patients with
two patients having sustained results and three having severe
side effects.74 In a prospective open study of 15 patients treated
with Etanercept 50 mg weekly, only three were considered as
responders75. Thus, the question is now raised: are tumour
necrosis factor-alpha blockers the ultimate alternative?76 It is
obvious that these drugs may be helpful, and sometimes have
a dramatic efficacy. Today, they are to be used in case of
failure of the standard very effective antibiotic association
of clindamycin and rifampicin. A face-to-face comparison of
anti-TNF drugs and this antibiotic association is highly
desirable.

Minor procedures. Local excisions and primary closure: When

the extent of skin involvement is limited, especially in case of


relapsing abscess and suppuration at the same location, a local
excision can be done. In patients with Hurleys stage 1I and a
permanent or recurring draining sinus, it is extremely helpful
with a minimum of distress for the patient as it can be done under
local anaesthesia and as ambulatory patient. It is unhelpful in
cases of many burrowing abscesses or in case of new lesions
appearing regularly in different locations.
Exteriorization and deroofing of tracts may be an alternative
therapy.79
Radical excision and healing with secondary intention or graft. It is
the best option, in fact the only option, in stage III. Such a wide
excision should be performed by a surgical team with a good
knowledge of HS. The extent of excision must be wide and deep
enough to remove all suppurative lesions and tracts, and also if
possible, all apocrine gland bearing skin to avoid recurrence.
Mapping of sinus tract with methyl blue intra-operatively is of
utmost importance. Spontaneous healing is frequently the best
solution. Primary closure is possible in the axilla but may lead to
limited mobility of arms. Secondary closure is done by graft or
flaps. Transitional colic derivation may be necessary to allow
healing in peri-anal location. Recurrences may occur either
because of insufficiently wide excision or because of the presence
of subclinical predisposing structural or functional abnormalities,
i.e. apocrine follicles in aberrant locations. The recurrence rate
after wide excision is less than 30%. A study of 72 patients treated
by excisional surgery of localized lesions reported a high
satisfaction of patient despite recurrences.80 Wide surgery does
not modify the spontaneous evolution of the disease which may
appear in another location not previously involved; it is not a
recurrence.
Lasers. CO2 laser excision is used in mild to moderate disease with

radiotherapy. Doses up to 8 gray have been delivered with


good results. Considering the spontaneous high risk of cancer,
especially skin cancers in gluteal and intergluteal locations,
this potentially carcinogenic treatment should be considered with
caution. Despite this risk, there are still advocates of radiotherapy
in HS.77

secondary healing. Superiority in comparison with a classic surgery


is a matter of debate.81,82 Laser depilation to prevent new lesions is
an investigative procedure in early mild HS.
Botulinum toxin injections have been used with claimed good
results.83
Photodynamic therapy has been disappointing. The deep lesions
of HS do not seem to be good targets for this kind of superficial
treatment.84

Surgical treatment78

Conclusion

It has to be performed by an experienced surgeon, aware of the


difficulties and failures which may occur in HS. The proper
identification of the entire lesion is important as remnant tissue
may facilitate recurrences. High-frequency ultrasound examination
of the skin and/or magnetic resonance imaging may be helpful.
The major clue to a good and permanent result is the mapping of sinus
tracts and fistulas, usually done during the surgical intervention.

HS is an orphan disease not because of its rarity it is frequent


but because of the absence of knowledge from physicians.
Treatments are not curative except wide surgery which can be
done only for very severe cases but entail good management;
using classical well-known treatments is often more useful than
the so-called innovative treatments, the balance of risk/benefits
of which are unknown today.

Radiotherapy. Several series of patients have been treated with

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Hidradenitis suppurativa, a review

CME questions for hidradenitis suppurativa (HS)


1. Establishing the diagnostic of HS relies upon:
a. Painful nodules
b. Location in the apocrine areas
c. Biologic markers of inflammation
d. Closed comedos
e. Chronicity
2. Epidemiology; HS:
a. has a prevalence of 1%
b. is equally prevalent in men and women
c. has a peak prevalence in young adults
d. is more prevalent in certain ethnic groups
e. Is more prevalent in big cities
3. Associated (risk) factors for HS:
a. Diabetes
b. Smoking
c. Overweight
d. family history of HS
e. Hyperandrogenism
4. Some lesions are highly specific for HS:
a. Draining sinus
b. Ulcerations
c. Folliculitis
d. Hypertrophic scars
e. lymph node enlargement
5. Cutaneous manifestations which may be present in HS:
a. Abscesses
b. Pyoderma gangrenosum-like ulcerations
c. Closed comedos
d. Pyogenic granulomas
e. Double ended comedos
6. Typical topography of HS includes involvement of:
a. Intermammary zone
b. lumbar location
c. Groins
d. Axillae
e. Inter gluteal furrow
7. Associated lesions in HS may include:
a. Retro-auricular cysts
b. Pilonidal sinus
c. Hypertrichosis
d. Dissecting folliculitis of the scalp
e. Relapsing common boils(furunculosis)
8. Evolution:
a. Progression from Hurleys class I to III is frequently
observed
b. Hurleys class II is characterized by alternation of acute
episodes and remissions
c. The severity may differ in various location of the same
patient
d. Fibrous scars are the hallmark of burn out disease
e. Individual nodular lesions have a mean duration of 3 days

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995

9. HS:
a. Quality of life is severely impaired even in moderate disease
b. Acute infectious complications is a major threat in HS
c. Squamous cell carcinoma may be a late complication in men
d. Fistula opening in the rectum is very unusual
e. Rheumatoid arthritis is frequently associated to HS
10. Pathology:
a. Involvement of apocrine glands is a late event
b. Sebaceous gland inflammation is observed in early lesions
c. The first pathological sign recorded is follicular occlusion
d. Early lymphocytic infiltration of the follicle is observed
e. Differential diagnostic from cutaneous Crohns disease
may be extremely difficult
11. Differential diagnostic of HS includes:
a. Actinomycosis
b. Infected Bartholins gland
c. Inflammed epidermoid cyst
d. Granuloma inguinale
e. Acne
12. Aetiology:
a. A family history of HS is recorded in 30% of patients
b. A specific Gram negative bacteria is responsible for perianal lesions
c. Hormonal disturbances are one of the major aetiological
factors
d. An impairment of the follicular innate immunity could be
a major aetiological factor
e. Obesity is both a risk factor and a severity factor
13. Severity of HS may be evaluated by:
a. Pain visual analogic scale
b. Sartorius score
c. Quality of life instruments
d. Frequency of flares in intermittent disease
e. Amount of fever and inflammation markers
14. Surgery of HS:
a. Limited excisions are useless
b. Radical wide excisionnal surgery is the only useful treatment in Hurleys class III
c. Packing is always necessary after incision of an abscess
d. Incision of a non fluctuating nodule is harmful and should
be avoided
e. Wide excisional surgery prevents extension of the disease in
distant areas
15. Antibiotherapy:
a. The choice of antibiotic should rely upon bacterial
identification
b. A very early use of antibiotics may prevent the evolution of
a nodule toward an abscess
c. Antibiotics may not primarily act through their antibacterial
properties
d. Clindamycin- Rifampicin association is effective in reducing
pain and suppuration

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996

e. Antibiotic treatments are only suspensive of the course of


the disease
16. Treatments:
a. Corticosteroids are effective to reduce painful nodules
b. Radiotherapy is a standard useful treatment in the long term
c. Zinc is useful in controlling moderate disease
d. Anti-androgens are pivotal in treatment of women
e. Dapsone has been used with good results in inflammatory
HS
17. Treatments:
a. None of the recommendation for treatment of HS is
evidence based
b. Anti-TNF biologics are becoming the first line medication
in HS
c. The superiority of anti-TNF upon antibiotics has been
demonstrated
d. Isotretinoin is the preferred drug in men
e. Clindamycin-rifampicin association may be useful prior
to surgery

Correct answers
1.
2.
3.
4.
5.
6.

a, b, e
a, c
b, c, d
a, d
a, b, d, e
a, c, d, e

7.
8.
9.
10.
11.
12.

a, b, d
c
a, c, d
a, c, d, e
a, b, c, d, e
a, d, e

13.
14.
15.
16.
17.

a, b, c, d
b, d
b, c, d, e
a, c, e
a, e

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78 Lapins J, Emtestam L. Surgery. In: Jemec G, Revuz J. Leyden J, eds,
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Journal compilation 2009 European Academy of Dermatology and Venereology

998

Revuz

Information on author
Jean Revuz was nominated intern of the Paris Hospitals in 1964. He did his resident medical
studentship in Paris, particularly in the Saint Louis Hospital as a student of Robert Degos.
In 1970, under the authority of Ren Touraine, Revuz co-founded with Touraine the dermatology
department of the Henri Mondor Hospital in Crteil. Henceforth, Revuz started to develop his
expertise in the area of serious dermatosis and drug reactions. He was nominated to a PhD in 1977 and
became a titular professor in 1990. In 1989, he became the head of the hospitals dermatology
department.
He is a member of numerous societies, e.g. the American Dermatology Academy, and was president
of the French Dermatology Society in 1995. Jean Revuz was also the president of the 20th World Congress
of Dermatology held in Paris 15 July 2002.
He is the author of more than 300 original publications and has likewise written a great number
of book chapters. Since 1971, he has been the prime mover of a team studying the secondary
cutaneous effects of medication. A great number of publications on serious drug reactions, in particular
the Lyell syndrome (Toxic Epidermal Necrolysis) and the organization of two congresses on this
subject have increased the reputation of his Crteil team.
For 10 years, he has been involved in taking care of patients with hidradenitis suppurativa and has
published several papers about epidemiology and treatment of this disease. Now retired from the
hospital, he has opened a private office in Paris devoted to this common but often ignored disease.

JEADV 2009, 23, 985 998

2009 The Author


Journal compilation 2009 European Academy of Dermatology and Venereology

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