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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1520_Библиотеки_им_академика_М_И_Перельмана
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78
N. Scott
After the plugging occurs, bacteria invade the apocrine system via the hair folli-
cles, become trapped beneath the keratinous plugs, and multiply rapidly in the apo-
crine sweat. The glands subsequently rupture and spread the infection to adjacent
apocrine and eccrine glands, further extending the tissue destruction and skin dam-
age. The signifi cance of bacterial infection in hidradenitis is controversial. Although
bacterial superinfection with streptococci , staphylococci , and coliforms is consid-
ered part of the pathogenesis of the disorder, cultures from lesions are frequently
sterile, and antibiotics are not curative.
Possible etiological factors for hidradenitis suppurativa include [ 2 ] :
• Deodorants , depilation , and shaving have been considered to be signifi cant fac-
tors; however, comparisons with age-matched controls showed no increased use
of these agents in hidradenitis sufferers.
• Obesity appears no more common in hidradenitis than in the normal population.
• High androgen levels have been suggested as an etiological factor in women.
However, there was no evidence of such an association when women with
hidradenitis were compared with controls matched for weight and hirsutism.
Moreover, high androgen levels as an etiological factor is diffi cult to reconcile
with hidradenitis being predominantly a female disease.
• Positive family history seems to be a common observation made in several stud-
ies suggesting possible autosomal dominant mode of transmission.
• Smoking is associated with hidradenitis; in one study, 88.9% of hidradenitis
patients smoked compared with 46% of a matched control group [ 3 ] .
6.3 Clinical Picture
More women than men are affected by hidradenitis suppurativa. Although any site
in either gender can be involved, there are signifi cant differences in gender distribu-
tion of skin involvement. The predominant female site for hidradenitis is the axillae,
while men more commonly have perianal involvement [ 2 ] .
Keratin plug
Apocrine Gland
Hair Follicle
Fig. 6.1 Keratin plug blocks
hair follicle; bacteria multiply
in apocrine gland which
bursts releasing bacteria –
secondary bacterial infection
leads to abscess formation
79
6 Hidradenitis Suppurativa
Diagnostic criteria proposed for hidradenitis include [ 2 ] :
Recurrent deep boils for >6 months in fl exural sites •
Onset after puberty •
Poor response to antibiotics •
Routine pus culture negative for pathogens •
Premenstrual exacerbation of boils •
The average number of painful eruptions in hidradenitis ranges between 1 per
year and 30 per month [
1 ] . The median is 2 per month. The average duration of a
single painful area is 6.9 days, but 62% of sufferers have a permanently painful area
that does not settle. Two-thirds fi nd that the painful swellings eventually burst –
one-third feel that most of their lesions do not ultimately burst. A simple staging
system for hidradenitis has been proposed (Box 6.2 ).
The average age of onset is 22 years. Average disease duration is nearly 19 years,
and maximum disease activity is in the fi rst 6 years or so. The proportion of females
with hidradenitis is signifi cantly lower in those over the age of 50 – suggesting that
hidradenitis may subside in women after the menopause [ 1 ] .
Hidradenitis causes a high degree of morbidity, with pain being the most impor-
tant contributor to this. Patients with hidradenitis fail to get appropriate recognition
for the severity of their disease [ 4 ] . Many of them seem to suffer in silence, as
refl ected in the name chosen by a hidradenitis self-help group “HIDE” (website:
http://webhome.globalserve.net.hidecan/ ) .
6.3.1 Perianal Hidradenitis
Anogenital involvement affects the groins, the pubis, the inner thighs with involve-
ment of the perineum, the buttocks, and the perianal skin (Fig. 6.2 ). As elsewhere,
blind painful “boils” form and lead to sinus track formation and “bridged” scarring.
In the majority of cases, the disease process is confi ned to the deep fascia; in more
advanced cases, the sinuses can dissect deep into muscle and fascia with a labyrinth
of tracks in advanced cases [ 6 ] . With chronicity, sinuses, scarring, and comedones
become apparent and characteristic. Epithelialization of the track is typical in
hidradenitis. The differential diagnosis of perianal hidradenitis includes perianal
Box 6.2: Hidradenitis: Hurley Staging System [ 5 ]
Stage I Solitary- or multiple-isolated abscess formation without scarring or
sinus tracts
Stage II Recurrent abscesses, single or multiple widely separated lesions, with
sinus tract formation
Stage III Diffuse or broad involvement across a regional area with multiple
interconnected sinus tracts and abscesses
80
N. Scott
Crohn’s disease and complex cryptoglandular anal fi stula disease. Hidradenitis sup-
purativa can involve anal canal, and if it does, usually tracks subcutaneously. In rare
cases, it crosses the sphincter.
6.3.2 Crohn’s Disease and Perianal Hidradenitis
Crohn’s disease and hidradenitis have been described as coexisting in the same
patient. In one series of 61 patients with hidradenitis, 24 (38%) also had a diagno-
sis of Crohn’s disease – ileal in one patient, ileocolic in 4, and colonic in 19 –
which predated the hidradenitis by 3.5 years on average. Perineal or perianal
hidradenitis was seen in all, but other sites were also involved by hidradenitis in 20
of this group [ 7 ] .
Both hidradenitis and Crohn’s disease can demonstrate histologically noncaseat-
ing epithelioid granulomas unrelated to foreign material [ 8 ] . It is unclear whether
the association between hidradenitis and Crohn’s disease is coincidental or whether
a small proportion of patients with hidradenitis suppurativa and epithelioid granu-
lomatous infl ammation represent a group of patients that is susceptible to systemic
granulomatous disease, including Crohn’s disease [ 8 ] .
Does this possible association between hidradenitis and Crohn’s disease affect
our management of perianal hidradenitis? Clearly, both conditions need to be treated
on their respective merits, but in addition two further steps might be considered in
selected cases:
MRI perineal imaging – marked thickening of the skin and induration of the •
subcutaneous tissues with no communication with the rectum and abscesses dis-
tinctly remote from the rectum and anus are not compatible with the fi stulous
tracks associated with Crohn’s disease and support a diagnosis of hidradenitis
suppurativa [ 9 ] .
Anti-TNF therapy with both infl iximab and adalimumab has been reported as •
being successful in healing diffi cult cases of hidradenitis suppurativa [
10, 11 ] .
Fig. 6.2 Stage III perianal
hidradenitis with broad
involvement across a regional
area – multiple
interconnected sinus tracts,
signifi cant cicatrization, and
persistent abscesses (prone
jackknife position)
81
6 Hidradenitis Suppurativa
6.4 Principles of Medical Management (Table 6.1 )
Conservative measures such as warm baths and nonsteroidal anti-infl ammatory
drugs may alleviate pain as well as infl ammation in hidradenitis. Although not
proven to be effective (and indeed nonresponse to antibiotics is a defi ning charac-
teristic of hidradenitis), antibiotics are regarded as the mainstay of medical
therapy [ 12 ] .
6.4.1 Long-Term Antibiotics
The average duration of painful nodules in hidradenitis is about 1 week – about the
duration of the average course of antibiotics. So the perceived response of hidraden-
itis to standard courses of antibiotics may be explained by the natural history of the
condition [ 4 ] .
By contrast, topical clindamycin cream has been used in hidradenitis with
some success. Similarly, long-term administration of erythromycin and tetracy-
cline has been used to treat the chronic hidradenitis and has been shown
to reduce the relapse rate. Initial treatments are usually oral antibiotics (tetracy-
cline, erythromycin, Augmentin) and topical antibiotics (clindamycin or
erythromycin).
Table 6.1 Evidence base for medical treatment of hidradenitis
Level of evidence Effi cacy
Local treatment
Clindamycin topical 1 Superior to placebo
Intralesional steroids 3 Effective
Antimicrobial washes 3 Unknown
Systemic treatment
Tetracycline 1 Equivalent to topical clindamycin
Cyproterone 1;2 No effect over estrogen therapy;
possibly effective over
antibiotics
Isoretinoin 2 Limited effi cacy
Clindamycin + rifampacin 2 Effective
Zinc salts 2 Effective
Cyclosporine 3 Effective
Dapsone 2 Effective
Systemic steroids 3 Possibly effective
Methotrexate 3 Weak to none
TNF- a inhibitors 2 Effective in some patients
Based on Table 1 in Gorovoy et al. [
11 ]
1 Randomized controlled trial evidence, 2 Retrospective, case-control, small therapeutic trials,
case series, 3 Case report, common practice
82
N. Scott
6.4.2 Estrogens
Oral contraceptive (e.g., 50 mcg ethynylestradiol) and the combination of estrogens
with 100 mg of cyproterone acetate have been used.
6.4.3 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. Retinoids are teratogenic, and preg-
nancy is prevented by the use of contraception. Approximately 40% of patients
show good response to retinoids.
Overall, both the evidence base (Table
6.1 ) and the effi cacy of medical therapies
in severe hidradenitis are poor, and patients with Stage II and III disease are usually
best helped by surgical excision of disease.
6.5 Hidradenitis Surgery: Principles
6.5.1 The Extent of Excision
The principle of surgical excision of hidradenitis suppurativa is that recurrence of
hidradenitis is minimized by removing all of the apocrine-bearing skin in the
affected area (Box 6.3 ). Thus the extent of the skin excision infl uences recurrence
rates more than a particular method of wound management. Recurrence after sur-
gery is likely if excision is inadequate or if there is an unusually wide distribution of
apocrine glands [ 13, 14 ] .
This principle (limited excision = hidradenitis recurrence) is illustrated in a series
of 31 patients treated surgically for hidradenitis and followed for a mean of
72 months [ 15 ] . Fourteen patients required reoperation for recurrent disease during
this period (Fig. 6.3 ):
Box 6.3: Right way - Wide Excision
There is a right way and a wrong way of performing surgery for Hidradenitis
patients. The right way is by wide excision with secondary intention heal-
ing or split-skin grafting. The wrong way is by incision or narrow excision
and primary closure
von de Werth [ 17 ]
83
6 Hidradenitis Suppurativa
After local drainage procedures, all six patients suffered recurrent hidradenitis •
within a median interval of 3 months.
After limited excision, the recurrence rate was 42.8%, within a median of •
11 months.
After wide excision, the recurrence rate was 27%, within a median of •
20 months [ 15 ] .
The site of the hidradenitis also has an infl uence on recurrence rates. Among 82
patients (118 excisions) treated by radical surgery, local recurrence varied with dis-
ease site – being low after axillary (3%) and perianal surgery (0%), and high after
inguinoperineal (37%) and submammary (50%) excision [ 16 ] . In this series, two
distinct patterns of recurrent disease were seen:
Confi ned to the immediate vicinity of the scar (ten patients), usually solitary and •
typically occurring in the groin creases or the posteroinferior margins after
inguinoperineal surgery – and requiring re-excision
Multiple lesions (nine patients), either adjacent to previous excision sites or a •
few centimeters away – characterized by widespread superfi cial lesions with lit-
tle morbidity
16
14
14
12
10
8
6
6
6
6
4
2
0
11
3
Incision
Local Excision Wide Excision
Number of Patients
Recurrence
n
Fig. 6.3 Hidradenitis recurrence and surgical technique (Based on Ritz et al. [ 15 ] )
84
N. Scott
6.6 Wound Management
Primary closure – Some algorithms of surgical excision for hidradenitis do suggest
excision and primary closure for limited axillary disease [
18 ] . However, most
authors have come to the view that if your hidradenitis excision can be closed by
primary suture, then you can also expect recurrence of the hidradenitis. Primary
closure is largely condemned because the limited degree of excision it involves
represents an inadequate therapy of this problem.
6.6.1 Wide Local Excision: Split Skin Grafting
Wide local excision and split-thickness skin grafting or fasciocutaneous fl aps can be
employed as the mainstay of treatment in patients with Stage II and Stage III disease
[ 5 ] . While split skin grafts can considerably speed up wound healing after wide
local excision, their use can also have some disadvantages including:
Painful and immobilized donor sites. •
Failure of the split skin graft to take particularly in inguinoperineal excision •
sites.
The fi nal size of the defect is much reduced by allowing the contraction of heal-•
ing by secondary intention.
6.6.2 Wide Local Excision: Healing by Secondary Intention
Wide excision of extensive hidradenitis necessarily produces extremely large
wounds. If left to heal by granulation, these wounds initially represent a consider-
able burden to the patient in terms of discomfort, discharge, and dressings in diffi -
cult areas – the axillae, the groins, and the perineum.
Primary closure, grafting, or fl aps have been extensively utilized but may be
associated with poorer results [ 19 ] – overall, healing by secondary intention is
thought to provide the best outcome.
In the past, a very useful technique of open wound management after hidradenitis
excision was the use of Silastic foam dressing. This dressing resulted in good cos-
metic outcome and was preferred by patients to the pain and immobilization of a split
skin donor site [ 20 ] . However, in the absence of Silastic foam dressing, nonadherent
dressings, silver-impregnated dressings, alginates, and hydrocolloid dressings are
useful and should be used according to wound conditions. It is important to keep the
wound clean and prevent infections to expedite healing. Local or systemic antibiotics
maybe required to control wound infection. Physiotherapy is needed to prevent con-
tractures and for early rehabilitation after wide excision of axillary skin [ 21 ] .
85
6 Hidradenitis Suppurativa
6.6.3 Perianal Hidradenitis Surgery
There are two compelling reasons for advocating surgical excision for perianal
hidradenitis. These are:
The relative lack of success of medical therapies for this chronic and socially •
isolating disease
The rare but persistent fi nding of malignant change in perianal hidradenitis, indi-•
cating that in some patients this disease is a premalignant condition that should
be excised [ 22 ]
The surgical management of perianal hidradenitis largely refl ects the manage-
ment of hidradenitis in other sites (Table 6.2 ). Typically, these patients are in their
mid-40s, and wide local excision is most commonly used to remove the perianal
disease. Clearance of perianal hidradenitis may necessitate excision laterally to a
distance of 10–12 cm and medially to the junction of true, thick perianal skin with
the thinner, rugose endoanal skin [ 16 ] .
As already pointed out, malignant change is not uncommonly seen in perianal
hidradenitis – usually squamous cell cancer – but there are also instances of
mucinous adenocarcinoma (Table 6.2 ). Colostomy diversion may be required in
some patients after perianal hidradenitis excision, but many patients can manage
their extensive perianal wounds without a stoma using a well-planned bowel
regimen.
Table 6.2 Summary of results of surgical treatment perianal hidradenitis
Endo et al. [ 23 ] Bocchini et al. [ 24 ]
Balik et al. [
25 ]
Number (Patients) 12 56
15
Gender 10 :2 52 :4 15
Mean age (years) 43 40 42.5
Site Perianal 21-Gluteal/17-
perineal/18-
inguinal
Gluteal/perianal/
inguinal
Management Wide excision-8 (4 not
treated)
All-wide excision All-wide excision
Malignant change 1 SCC; 1 mucinous
adenoca
1 SCC 1 SCC
Colostomy Nil 23 Colostomies (41%) 1 Colostomy
Time to healing Nongrafted (3) N/A Nongrafted 10(7–17)
weeks
Nongrafted (11) 12.2
(9.5–22) weeks
Skin graft (5) N/A Skin graft 6(3–9)
weeks
Delayed skin graft (2)
8 weeks
Rotation fl ap (2)
2 weeks
Recurrence 0% 1(1.8%) 0% at 5 years
SCC squamous cell carcinoma, adenoca adenocarcinoma, N/A not available
86
N. Scott
Wide excision of perianal hidradenitis is a very successful therapy with extremely
low rates of subsequent recurrence. Although split skin grafting [ 26 ] can be suc-
cessfully employed after wide excision of perianal hidradenitis, others report the
complete failure of this technique after perianal excision [
27 ] . Bilateral excision of
extensive disease leaves a large and uncomfortable wound; normal activities are
markedly restricted until the wound heals. Sequential unilateral excision starting
with the more affected side is a reasonable alternative.
In any case, it can be argued that better cosmetic results are obtained by allowing
healing by secondary intention (Fig.
6.4 ) contraction to reduce the size of the fi nal
defect. Overall, healing of perianal hidradenitis excision wounds with split skin
grafts can be expected to take place over 6–8 weeks. By contrast, healing by granu-
lation takes on average 10–12 weeks – but may extend to 5 or 6 months in individual
patients. However, wide excision cures the large majority of patients of perianal
hidradenitis with a very low rate of subsequent recurrence.
a
Fig. 6.4 Wide excision of
Stage III perianal
hidradenitis and healing by
secondary intention –
without colostomy diversion.
( a ) Initial extent of excision,
not preservation of skin at
anal margin. ( b ) Healthy
granulation tissue as the
wound heals by second
intention. ( c ) Contraction of
the wound markedly reduces
the size of the fi nal defect
Key Points
Hidradenitis suppurativa is common in 1–2% of the population and is asso-•
ciated both with cigarette smoking and a familial predisposition.
Perianal hidradenitis is largely a disease of men. •
Effective medical therapies include long-term topical clindamycin. •
Excisional surgery that allows primary closure is very likely to lead to •
recurrence.
Wide excision is the treatment of choice – either with split skin grafting or •
healing by secondary intention.
Long-standing perianal hidradenitis can lead to the development of •
squamous cell carcinoma.
87
6 Hidradenitis Suppurativa
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Dermatol Venereol. 2000;14:389–92.
2. Brown TJ, Rosen T, Orengo IF. Current concepts – hidradenitis suppurativa. South Med J.
1998;91(12):1107–14.
3. Konig A, Lehmann C, Rompel R, Happle R. Cigarette smoking as a triggering factor of
hidradenitis suppurativa. Dermatology. 1999;198:261–4.
4. Von der Werth JM, Jemec GBE. Morbidity in patients with hidradenitis suppurativa. Br J
Dermatol. 2001;144:809–13.
5. Menderes A, Sunay O, Vayvada H, Yilmaz M. Surgical management of hidradenitis suppura-
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6. Mortimer PS, Lunniss PJ. Hidradenitis supprativa. J R Soc Med. 2000;93:420–2.
7. Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. The differential diagnosis and
comorbidity of hidradenitis suppurativa and perianal Crohn’s disease. Int J Colorectal Dis.
1993;8(3):117–9.
8. Roy MK, Appleton MA, Delicata RJ, Sharma AK, Williams GT, Carey PD. Probable associa-
tion between hidradenitis suppurativa and Crohn’s disease: signifi cance of epithelioid granu-
loma. Br J Surg. 1997;84(3):375–6.
9. Kelly AM, Cronin P. MRI features of hidradenitis suppurativa and review of the literature. AJR
Am J Roentgenol. 2005;185:1201–4.
bc
Fig. 6.4 (continued)
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