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338 The ASCRS Manual of Colon and Rectal Surgery
B. Hidradenitis Suppurativa
Background
• Hidradenitis suppurativa is a cutaneous condition that involves
skin containing apocrine sweat glands.
• Areas of the body where this often occurs include the perineum,
the axilla, and the groin. It presents initially as an abscess, but
typically is recurrent in the affected area and ultimately can
lead to severe scarring and disability for the patient.
Incidence and Etiology
• One in every 300 individuals may be affected in some way.
• African-Americans seem to be affected more often than Caucasians, and perianal disease seems to be more common in males.
• Almost all patients present after puberty and before the age of
40, implicating hormones and the development of secondary
sexual characteristics as causative.
• Other endocrine associations include diabetes mellitus, hypercholesterolemia, and Cushing’s disease.
• Obesity has been implicated as a predisposing factor presumably
from shearing forces in the affected areas.
• Perianal hidradenitis affects males twice as often as females,
but hidradenitis in all locations may be more common in
females and African-American persons.
• Fortunately for sufferers of perianal hidradenitis, it seems to
recur less often after surgical treatment (<0.5%) than does
inguinal-perineal disease (37–74%).
Bacteriology
• Wound cultures from hidradenitis patients have grown Staphylococcus epidermidis , Escherichia coli , Klebsiella , Proteus , alpha
Streptococcus , anaerobic bacteria, and diptheroids, although
negative cultures are common.
Pathogenesis
• Most authors agree that hidradenitis suppurativa originates
from obstruction of apocrine sweat glands by keratin. However,
15. Pilonidal Disease and Hidradenitis Suppurativa 339
it is unknown why this occurs in some people and not in others
(females, African-Americans, etc).
• These glands secrete a milky, odorless fl uid that only becomes
malodorous after it interacts with bacteria on the skin. The
apocrine glands secrete into the hair follicle as opposed to
directly onto the skin like eccrine sweat glands.
• The function of apocrine secretion is unknown. Nevertheless,
obstruction leads to secondary bacterial infection and rupture
of the gland into the dermis and subcutaneous tissue, thus
causing cellulitis, abscess, and draining sinuses.
• This process then leads to the characteristic “pit-like” scars
from chronic fi brosis of the destroyed glandular unit.
Differential Diagnosis
• Cutaneous infections such as furuncles, carbuncles, lymphogranuloma venereum, erysipelas, epidermoid or dermoid cysts, and
tuberculosis can be particularly troublesome.
• In particular, it must be distinguished from other fi stulizing
or sinus-forming processes of the perineum. Crohn’s disease
typically affects the anus and rectum with fi stulas arising from
the dentate line or higher in the rectum.
• Hidradenitis does not affect the rectum, because apocrine
glands only exist in the lower two-thirds of the anal canal and
do not penetrate into the sphincter complex. Thus, patients will
not have sinus or fi stula tracks to or from the rectum.
• Fistulas from hidradenitis should only connect areas of involved
skin, and not penetrate the anal sphincters
• Several cases of squamous cell carcinoma in chronic hidradenitis
wounds have also been published.
– The association seems to be rare with affected patients,
who usually have had untreated disease for longer than
20 years.
Treatment: Initial
• Hidradenitis suppurativa typically presents with pain, erythema,
and swelling in the affected area. Patients with cellulitis and
no defi nable clinical abscess may be successfully treated with
antibiotics that cover skin fl ora, such as Staphylococcus
species, over 1–2 weeks.
340 The ASCRS Manual of Colon and Rectal Surgery
• The safest course of action with any patient who presents with
an obvious abscess is incision and drainage.
• Eighty-three percent of patients will have recurrent localized sepsis
of some sort after initial incision and drainage or limited excision.
Treatment: Chronic
• Chronic disease is simply any hidradenitis disease persisting or
recurring after initial treatment. This could present as recurrent
abscesses, nodules, sinuses, fi stulas, cellulitis, or any combination
of these problems.
• Unless the surgeon excises all this skin, the patient will technically
be at risk for recurrence, although not every patient eventually
goes on to radical excision.
• Excision with healing by secondary intention is probably the
most widely used surgical treatment.
• Only the grossly involved apocrine bearing skin (but all of it)
in the perianal area should be excised full thickness into the
uninvolved gluteal fat.
– This method is simple and almost never requires fecal
diversion. It also allows completion of the procedure as an
outpatient. Perioperative antibiotics are unnecessary.
– Patients with large areas of involvement may undergo
staged excision. The extent of excision should remain
outside the anal verge as long as there is no obvious
involvement or history of involvement in the anal canal.
• Patients with chronic disease, extensive scarring, and sinus
tracts rarely respond to conservative measures. The gold standard
of care remains wide excision of all skin bearing involved
apocrine glands. Reconstruction then can follow a number of
paths – unroofi ng of sinus tracts with or without marsupialization, cutaneous fl ap closure, myocutaneous fl ap closure, or
excision and simple healing by secondary intent.
• Patients who might benefi t from diversion are those who cannot ake care of their wounds long term and those who have both
hidradenitis and Crohn’s disease, although this is rarely needed.
Summary
• The algorithm in Fig. 15.7 depicts our suggested approach to
treating patients with perianal hidradenitis suppurativa. Patients
15. Pilonidal Disease and Hidradenitis Suppurativa 341
Initial abscess
Incision and drainage
Resolution
Localized disease to
perianal region
Excision (staged, if necessary) with
healing by secondary intent
Fig. 15.7. Perianal hidradenitis suppurativa algorithm.
Sitz baths/ hygiene
Multiple recurrences/
patient desires surgery
Extensive disease with
scarring out onto buttocks
Cutaneous flap Myocutaneous flap
who present initially with an acute abscess, and a history and
examination consistent with hidradenitis, should have incision
and drainage, ideally in an offi ce setting.
• Physicians should reserve antibiotics for those patients with a
component of cellulitis as discussed above, or those who are
immunocompromised.
• It is important to rule out other causes of perianal sepsis in the
early stages of the disease, such as Crohn’s disease or perirectal abscesses from a cryptoglandular source.
• For those patients with chronic and/or recurring disease, we
proceed to defi nitive excision, as long as the diagnosis is not in
doubt and we have exhausted the simpler alternatives.
• Flap procedures are reserved for patients with extensive scarring
and tissue damage out onto skin distant from the anus, such as
the buttocks.
• Even relatively large open wounds around the anus heal
remarkably well in the absence of Crohn’s disease and other
infl ammatory, malignant, or infectious processes, compared
with how similar wounds typically heal in other areas of the
body. Because of this, it is usually not necessary to use fl aps
after skin excisions for hidradenitis around the anus, especially
when using a staged approach.
342 The ASCRS Manual of Colon and Rectal Surgery
• If circumferential disease is present and requires excision, we
excise half of the involved perianal skin down to subcutaneous
fat and allow the wound to heal by secondary intent, which
may take up to 3 months. We excise the other half after complete
healing of the fi rst wound.
• If circumferential excision of perianal skin is considered in a
single procedure, we take care not to excise the skin at or inside
the anal verge. This diminishes the risk of anal stricture. For
patients whose disease does not extend out more than 5 or 6 cm
from the anal verge, this approach works very well.
• We consider a fl ap-based procedure for those patients with
much wider involvement extending out onto the buttocks.
16. Perianal Dermatology
and Pruritus Ani
A. Introduction
• Perianal skin is subject to virtually all of the diseases that affect
skin in other areas of the body. The differential diagnosis of
perianal skin is presented in Table 16.1 . This list includes a
variety of diagnoses, which almost never present as isolated
perianal disease, but there are common diseases such as
psoriasis that may present in isolation without obvious ties to
other areas of the body unless a careful search is made.
• Successful treatment of perianal disease requires accurate
diagnosis to eliminate diseases that have specifi c cause and
treatment (e.g., psoriasis, candida, Bowen’s disease).
• Recognition of important treatable causes requires a disciplined, organized approach to diagnosis with frequent use
of biopsy.
• The importance of complete, accurate evaluation is emphasized
by a St. Louis University series in which a study of 209 patients
with the presenting symptom of pruritus over a 2-year period
revealed that 75% of patients had coexisting anal or colorectal
pathology. The diagnoses included 11% with rectal cancer,
6% with anal canal cancer, and 2% with colon cancer, although
the majority of patients had hemorrhoids or fi ssure.
B. Defi nitions
• Pruritus ani is a term of Latin derivation, which means itchy anus.
D.E. Beck et al. (eds.), The ASCRS Manual of Colon and Rectal Surgery, 343
DOI: 10.1007/978-0-387-73440-8_16, © Springer Science + Business Media, LLC 2009
344 The ASCRS Manual of Colon and Rectal Surgery
Table 16. 1. Differential diagnosis of anal dermatoses.
Infl ammatory disease Nonsexual infectious disease
Pruritus ani Pilonidal disease
Psoriasis Hidradenitis suppurativa
Lichen planus Fistula-in-ano
Lichen sclerosis et atrophicus Crohn’s disease
Atrophoderma Tuberculosis
Contact (allergic) dermatitis Actinomycosis
Seborrheic dermatitis Herpes zoster
Atopic dermatitis Vaccinia
Radiation dermatitis Fournier’s gangrene
Behçet’s syndrome Tinea cruris
Lupus erythematosus Candidiasis
Dermatomyositis “Deep” mycoses
Scleroderma Amebiasis cutis
Erythema multiforme Trichomoniasis
Familial chronic pemphigus Schistosomiasis cutis
(Hailey-Hailey) Bilharziasis
Pemphigus vulgaris Oxyuris (pinworm)
Cicatricial pemphigoid Creeping eruption (larva migrans)
Larva currens
Cimicosis (bed bugs)
Pediculosis (lice)
Scabies
Sexually transmitted disease Premalignant and malignant disease
Gonorrhea Acanthosis nigricans
Syphilis Leukoplakia
Chancroid Mycosis fungoides
Granuloma inguinale Leukemia cutis
Lymphogranuloma venereum Basal cell carcinoma
Molluscum contagiosum Squamous cell carcinoma
Herpes simplex Melanoma
Condyloma acuminate Bowen’s disease (AIN)
Extramammary Paget’s disease
Source : Modifi ed from Corman.
• Pruritus ani has been classifi ed into primary and secondary.
The primary form is the classic syndrome of idiopathic pruritus
ani, whereas the secondary form implies an identifi able cause
or a specifi c diagnosis.
• Accurate description of the morphology of skin lesions can aid in
the diagnosis and follow-up of patients with pruritic complaints.
16. Perianal Dermatology and Pruritus Ani 345
– Macules are fl at spots.
– Papules are elevated circumscribed solid lesions, raised
spots.
– Vesicles are separations of the epidermis and dermis
fi lled with serum.
– Bulla are larger vesicles or blisters.
– Pustules contain pus.
– Ulcers are surface lesions with loss of continuity of the skin
and may result from rupture of vesicular lesions, infection,
or trauma.
– Intertrigo is infl ammation seen between two opposing skin
surfaces, often the result of mixed bacterial, fungal infection
associated with moisture, obesity, and poor hygiene.
C. Physiologic Considerations
• Itch is a surface phenomenon mediated by pain fibers in
the epidermis that may have a lower threshold for stimulation
than pain.
• Itch receptors may be located more superfi cially than those
dedicated to pain. Because receptors are superfi cial, innocuous,
nondamaging stimuli such as wearing wool, or other minor
mechanical stimuli may induce itching.
• In addition to histamine, kallikrein, bradykinin, papain, and
trypsin experimentally produce itching, but these substances
do not respond to blockade with histamine antagonists such as
diphenhydramine, hence topical antihistamines are not always
effective against itching.
• The phenomenon of hyperesthesia with chronic pain may have
a parallel with itching, whereas minimal stimulation of the skin
may induce itching; scratching with subsequent injury may
produce an enlarging patch of itchy skin. Scratching produces
inadequate feedback to inhibit itching; more scratching occurs
with cutaneous injury, which provides an additional stimulus
to scratch in a self-defeating loop.
• Itching attending the healing of surgical wounds and scars
probably results from the combination of histamine release,
release of other kinins and prostaglandins involved in the
infl ammatory phase of healing, and regeneration of nerves that
may be thinly myelinated in immature scars.
346 The ASCRS Manual of Colon and Rectal Surgery
• Antihistamines, topical anti-infl ammatory agents (steroids),
topical anesthetics, and aloe preparations (prostaglandin inhibitors)
all have benefi cial effects on the itching of healing wounds.
D. Etiology of Pruritus
• Because pruritus is a symptom that may have protean causes, it
is useful to consider diagnoses that have been associated with
pruritus ani. Table 16.2 is a list of diagnoses and conditions
modifi ed from Stamos and Hicks.
Localized Itch Syndromes
• Notalgia paresthetica is a defi ned syndrome with itching or pain
of the upper mid back to either side of the scapular region.
• Dermographism has been reported as a cause of anogenital
pruritus. It is not unreasonable to propose that the idiopathic
form of pruritus ani may be a related disorder, and that the skin
changes are the sole result of skin trauma. The effectiveness
of the anal tattooing procedures, discussed later, lends some
support to this hypothesis.
Fecal Contamination
• Good evidence supports fecal contamination as one cause of
symptoms.
• Smith and colleagues in a rigorous study of 75 patients with
pruritus found that half of their patients had poorly formed
stools and 41% of their patients complained of soiling from
daily to several times a week. Seepage of liquid and mucous
was believed to be an important factor in the etiology of the
symptoms. Coffee was demonstrated to lower anal resting
pressure in 8 of 11 patients.
Viral Infection
• Condylomata acuminata are a common cause of itching, but
the diagnosis is easily recognizable and should not be confused
with idiopathic pruritus ani.
16. Perianal Dermatology and Pruritus Ani 347
Table 16. 2. Proposed etiologies of idiopathic pruritus ani.
Anatomic factors Obesity, deep clefts, hirsutism, tight clothing
Anorectal disease Fissure, fi stula, tags, prolapsing papilla,
hemorrhoids, mucosal prolapse, sphincter
insuffi ciency, deforming scars
Antibiotics
Contact dermatitis Chemicals in topical preparations, toilet paper,
wet wipes, alcohol, witch hazel, “caine” anesthetics,
fecal soiling
Dermatoses Psoriasis, seborrheic dermatitis, atopic dermatitis,
lichen planus, lichen simplex, LS, dermographism
Diet Coffee (caffeinated and decaffeinated), chocolate,
spicy foods, citrus fruits, tomatoes, beer, dairy
products, vitamin A and D defi ciencies, fat substi-
tutes, consumption of large volumes of liquids
Diarrhea Infectious diarrhea, irritable bowel syndrome,
Crohn’s disease, ulcerative colitis
Drugs Quinidine, colchicine, intravenous steroids
Gynecologic conditions Pruritus vulvae, vaginal discharge of infection
Idiopathic
Infection Viruses: herpes simplex, cytomegalovirus,
papillomavirus; bacteria: S. aureus , beta hemolytic
strep, mixed infections; fungi: dermatophytes,
Candida species; parasites: pinworms, scabies,
pediculosis; spirochetes: syphilis
Neoplasms Bowen’s disease (AIN), extramammary Paget’s
disease, squamous cell carcinoma variants, secreting
villous tumors
Personal hygiene Poor cleansing habits, over-meticulous cleansing
producing mechanical trauma, use of soaps
Psychogenic/neurogenic Anxiety, neurosis, psychosis, neurodermatitis,
neuropathy, “itch syndromes”
Radiation Radiation dermatitis, sphincter compromise or
leakage caused by radiation proctitis
Systemic disease Jaundice, diabetes mellitus, chronic renal failure,
iron defi ciency, thyroid disorders, lymphoma,
polycythemia vera
Source : Modifi ed from Stamos and Hicks, 1998.
• Herpes syndromes are usually accompanied by pain rather than
itching and the clinical course is accompanied by a characteristic eruption consisting of red macules, which progress to
vesicles that rupture, ulcerate, and may become secondarily
infected. Culture or biopsy shows specifi c diagnostic fi ndings.
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