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20 Anal Conditions: Pruritus Ani
161
E. Infectious causes of pruritus ani include bac-
terial, fungal, viral and parasitic infections.
• Common bacterial causes of pruritus ani
include erythrasma, gonorrhea, and
syphilis.
– Corynebacterium minutissimum causes
erythrasma which affects the perianal
area, axilla, thighs, and toe-web spaces.
A classic, large pink-reddish patch is
seen initially which eventually turns
brown (Figure20.3a). Under an ultraviolet lamp, the lesions appear with a
coral to salmon uorescence from the
porphyrin production made from the
bacteria. Treatment is erythromycin
250mg four times a day for 10days.
– Patients who present with tenesmus, puru-
lence, proctitis, in addition to pruritus,
b
Fig. 20.3 Bacterial infections. (a) Erythrasma. Courtesy of Lee Smith, MD; (b) Purulence seen with gonorrhea infec-
tion. Courtesy of Lee Smith, MD; (c) Primary chancre of syphilis. Courtesy of Lee Smith, MD
162
should be tested for gonococcal infection
(Figure 20.3b). A swab should be done
and placed on Thayer-Martin media. Anal
gonorrhea is treated with ceftriaxone
250mg IM plus azithromycin 1g PO.
– Syphilis often presents as a painless
chancre, starting as a papule that eventually ulcerates (Figure20.3c). In contrast to syphilis, painful ulcers in the
perianal region are usually associated
with herpes and chancroid. Syphilis is
caused by the spiral-shaped bacterium
Treponema pallidum. These spirochetes
can be seen on dark-eld microscopy
from scrapings obtained at the base of
the lesion. Alternatively, serologic
screening can be done with a nontreponemal test. Treatment is a one-time dose
of penicillin G 2.4 million units IM.
• Pruritus ani from a fungal infection presents with a markedly erythematous rash
(Fig.20.4). This condition is more common in patients with diabetes mellitus,
obesity and immunocompromised states.
Histopathology reveals hyphae of a fungus seen with a potassium hydroxide
preparation. These patients often respond
to topical nystatin 100,000units/g two to
three times a day.
• Viral etiologies of pruritus ani include
herpes (HSV) and condyloma.
– HSV infection often presents as painful,
scattered lesions including ulcers and vesicles (Figure20.5a). A viral culture taken
from the base of the ulcer or from vesicular uid is usually diagnostic. Treatment
of an acute episode is acyclovir 800mg
three times a day for two days or valacyclovir 500mg PO three times a day. For
patients with frequent recurrences, acyclovir 400 mg twice daily or valacyclovir
500mg daily has been advocated.
– Large anal condylomata can cause pru-
ritus and usually require excision and/or
fulguration in the operating room
(Figure20.5b).
• Pinworm (Enterobius vermicularis) is a
parasitic roundworm that can lead to pruritus ani. Gravid female pinworms migrate
B. L. Bello and K. Umanskiy
Fig. 20.4 Severe fungal infection. (With permission
from Smith L.Perianal Dermatologic Disease. In: Gordon
PH and Nivatvong S, editors. Principles and Practices of
Surgery for the Colon, Rectum, and Anus. third ed. 2007
© Informa Healthcare publishing)
from the anus and eggs are left on the
perianal folds causing irritation. This condition, more commonly affecting children, is notable for nocturnal itching.
Scotch tape applied to the perianal region
can reveal the eggs of the pinworm.
Lactophenol can be used to enhance the
slide (Fig. 20.6). Treatment is mebendazole 100mg PO as a single dose.
F. The most common dermatologic conditions
associated with pruritus ani are discussed
below.
• Psoriasis presents with erythema and
sharply dened boundaries with or without
the typical scaling (Fig.20.7). Patients with
psoriasis will characteristically have lesions
affecting the groin, genitalia, intergluteal
cleft, axilla, and umbilicus. Treatment is
usually a low to mid-potency topical steroid.
Tacrolimus can also be used.
20 Anal Conditions: Pruritus Ani
a
163
Fig. 20.6 Eggs of Enterobius vermicularis. (With per-
mission from Smith L.Perianal Dermatologic Disease. In
Gordon PH and Nivatvong S, editors. Principles and
Practices of Surgery for the Colon, Rectum, and Anus. 3rd
edn. 2007 © Informa Healthcare publishing)
b
Fig. 20.5 Viral infections. (a) Herpes lesions Courtesy of
Lee Smith, MD; (b) Anal condylomata. Courtesy of Lee
Smith, MD
• Lichen simplex chronicus is a condition
that can result from chronic diarrhea.
Inammation in the perianal area results in
thickened (lichenied) and cracked, excoriated skin (Fig.20.8). Treatment is focused
on controlling the frequency of bowel
movements. Loperamide and silver sulfadiazine can be used with addition of low
dose hydrocortisone for more severe cases.
• Lichen sclerosis (formerly lichen et atro-
phicus) presents mainly in women with a
thinning and wrinkling of the perianal
skin, also known as a “cigarette-paper”
appearance (Fig. 20.9). This also classically affects the labial skin and perineum.
Lichen sclerosis may be associated with
squamous cell carcinoma. Thus, the
affected area should be examined at least
annually and a biopsy should be considered for any suspicious lesions. Treatment
is a topical glucocorticoid like clobetasol
propionate 0.05% for 6–8 weeks.
Tacrolimus has also been used for this
condition.
• Contact dermatitis is a result of a mechanical or chemical irritant that may act as an
allergen. Some soaps, cleansers, alcohol
and feces can cause macular erythema,
hyperkeratosis, and ssuring (Fig.20.10).
Patch testing by an allergist or dermatologist can be useful to determine if there is
an inciting allergen, especially in severe
or refractory contact dermatitis. Treatment
is Sitz baths with or without vinegar, low
dose hydrocortisone, oral steroids, or
antihistamines.
• Pruritus ani with thickened skin and leathery patches may suggest atopic dermati-
164
B. L. Bello and K. Umanskiy
Fig. 20.7 Psoriasis around anus and intergluteal cleft.
Courtesy of Lee Smith, MD
tis. This is often hereditary and presents at
an earlier age than other causes of pruritus. In addition to the anus, this can be
seen in the neck, antecubital, and popliteal
fossas. Treatment is with a topical barrier
like petroleum jelly, and anti-inammatory drugs and antihistamines.
G. Systemic causes of pruritus ani include diabe-
tes mellitus, leukemia and lymphoma, cholestasis, thyrotoxicosis, and psychiatric
illnesses. Anxiety, stress, fatigue, and obsessive compulsive tendencies have been shown
to play a role. Often a generalized pruritus is
noted in these systemic conditions. Treatment
should focus on disease-specic interventions.
Systemic medications such as antibiotics (tetracycline and colchicine), quinidine, and peppermint oil have been implicated as well.
H. Local irritants are often the cause of anal
itching. Several of these have been discussed
above under contact dermatitis. Fecal seep-
Fig. 20.8 Lichen simplex chronicus. (With permission
from Finne CO, Fenyk JR.Dermatology and Pruritus Ani.
In: Beck DE, Roberts PL, Saclarides TJ, et al., editors.
The ASCRS Textbook of Colon and Rectal Surgery. 2nd
edn. 2011 © Springer publishing)
Fig. 20.9 Lichen sclerosis. Courtesy of Lee Smith, MD
age is the most common irritant responsible
for pruritus. This is further exacerbated by
excessive soaps and wiping in an effort to
clean the perianal region. Anal seepage can
be exacerbated by certain foods that can alter
the pH of the stool or lower sphincter tone. In
addition, ill-tting, synthetic clothes and top-
20 Anal Conditions: Pruritus Ani
Fig. 20.10 Contact dermatitis. (With permission from
Smith L. Perianal Dermatologic Disease. In Gordon PH
and Nivatvong S, editors. Principles and Practices of
Surgery for the Colon, Rectum, and Anus. 3rd edn. 2007
© Informa Healthcare publishing)
ical creams can trap moisture around the
anus. Treatment involves removing the
offending agent, keeping the area dry (cotton
ball or folded cotton gauze), and avoiding
further trauma to the skin. In the case of fecal
seepage, bulking agents are an effective rst
line treatment.
I. Colorectal and anal specic causes of pruri-
tus ani include hemorrhoids, ssure, stulain- ano, dysplasia, and malignancy. 6–11%
of patients with pruritus ani can have an
underlying cancer. This underscores the
importance of considering an endoscopy as
part of the diagnostic workup. The mainstay
of treatment is addressing the primary
cause.
J. Despite extensive work up, no clear etiology
of pruritus ani can be identied in up to 25%
of patients. These cases are classied as idio-
pathic, or primary, pruritus ani and are considered as a diagnosis of exclusion.
165
K. The majority of patients with either second-
ary or primary pruritus ani will benet from
simple, general principles including improving anal hygiene, removing any potential
inciting agents, food education, and improving bowel habit. These interventions can be
effective in up to 90% idiopathic cases.
• Inciting agents: Any inciting factors,
mechanical or chemical irritants, trauma,
and scratching should be avoided.
• Hygiene: Sitz baths without additives
after defecation often helps keep the perianal clean. Bidets are becoming more
popular as an alternative. Patients should
be counseled to avoid soaps, scrubbing,
and aggressive wiping. Excessive moisture can cause hygiene problems. Blotting
with damp toilet paper should be used
instead of a moist wipe. Using a hair dryer
on the lowest setting or dabbing with a
towel is also benecial. Light cotton as
undergarments should be used instead of
tight tting, synthetic underwear. A dry
cotton ball or gauze placed at the anus can
be used to limit moisture in the area. As a
general rule, topical creams should be
avoided initially as they may trap
moisture.
• Food education: Patients may benet
from avoiding coffee, cola, beer, tomatoes, chocolate, tea, citrus, and lactose
containing foods.
• Bowel habit: High ber diet and bulking
agents are helpful to in absorbing water
from stool, in turn decreasing fecal seepage. Antidiarrheals such as loperamide or
atropine/diphenoxylate are recommended
if needed.
If following these simple, general principles is not successful after four to six
weeks, a short-course trial of a lowpotency topical steroid (1% hydrocortisone) can be tried twice a day for two
weeks. This should be tapered off using a
barrier cream containing zinc oxide to
prevent skin atrophy.
L. Clinicians should be prepared to manage
refractory pruritus ani if there is no resolu-
166
B. L. Bello and K. Umanskiy
tion of symptoms despite previous treatment.
Repeating a thorough history may identify an
inciting event that may have not been identied initially. Journals with foods and/or timing of symptoms can demonstrate a temporal
relation to onset of symptoms. A biopsy and
endoscopy should be performed if they were
not done at the initial evaluation. Similar to
initial evaluation, the focus should be on
nding an underlying cause. These patients
will need to be counseled that refractory pruritus ani may be a chronic condition requiring a long-term treatment plan and their
expectations need to be set that treatments
are aimed at improving symptoms rather than
complete resolution. Capsaicin, anal tattooing, and tacrolimus are effective in the management of refractory pruritus ani.
M. Capsaicin is chili pepper extract and works
by depleting substance P and damaging
C-ber terminals, the bers that mediate
itch signaling (Fig. 20.2). A temporary
burning sensation replaces the overwhelming urge to scratch. Lysy etal. (2003) performed a randomized, control trial on
capsaicin versus menthol as placebo in
patients with idiopathic refractory pruritus
ani. Patients kept a 28day symptom diary
and scored their symptoms on a 1 to 5 point
scale. Capsaicin 0.006% for four weeks was
shown to improve these itching and burning
sensation scores in 75% of patients when
compared to placebo.
N. Anal tattooing involves the intradermal injec-
tion of methylene blue which destroys dermal nerve endings. The solution has been
modied to avoid skin necrosis which was
reported in up to 25% patients. The perianal
area is injected with 10ml of 1% methylene
blue plus 5 ml normal saline plus 7.5 ml
0.25% bupivacaine with epinephrine plus
7.5ml of 0.5% lidocaine. The tattoo disappears in about three to four weeks. Patients
can sometimes have prolonged numbness
around the area or see bluish urine initially.
Transient fecal incontinence has also been
described. In several retrospective studies,
Table 20.1 Summary of Studies on Anal Tattooing
Number
of
Author and Year
Eusebio etal.
1990
Farouk and
Lee 1997
Mentes etal.
2004
Sutherland
etal. 2009
Samalavicius
etal. 2012
Table 20.2 Summary of Studies on Tacrolimus
Author and
Year
Suys
2012
Ucak
etal.
2013
patients Key ndings
21 100% had improvement
in symptoms
6 83% had improvement in
symptoms;
50% needed a second
injection
30 93% had improvement in
symptoms (5 pts required
an additional treatment);
76% had complete
resolution at 12months
49 96% had improvement in
symptoms (4 pts required
an additional treatment);
57% had complete
resolution at 8weeks
10 100% had improvement
in symptoms at 4weeks;
20% had complete
resolution at 5years
Number of
patients Key ndings
21 68% had improvement in
symptoms at 2weeks
32 80% had improvement in
symptoms at 4weeks;
18.75% had complete
resolution at 18weeks
approximately 80–100% of patients had
some improvement, while 20–80% patients
had complete resolution of pruritus ani on
long-term follow up (Table 20.1). A second
injection may sometimes be helpful.
O. Tacrolimus 0.03–0.1% is a non- corticosteroid,
macrolide anti-inammatory and has recently
been studied in small series. This agent may
be a good alternative to topical steroids or as
a replacement when tapering off steroids to
help avoid skin atrophy. Two studies have
shown an improvement in itch intensity, itch
frequency, and Dermatology Life Quality
Index (DLQI), a quality-of-life questionnaire
(Table20.2).
20 Anal Conditions: Pruritus Ani
167
Suggested Reading
Eusebio EB, Graham J, Mody N.Treatment of intractable
pruritus ani. Dis Colon Rectum. 1990;33(9):770–2.
Farouk R, Lee PW.Intradermal methylene blue injection
for the treatment of intractable idiopathic pruritus ani.
Br J Surg. 1997;84(5):670.
Gaertner WB, Melton GB. Dermatology and pruritus
ani. In: Steele SR, Hull TL, Read TE, Saclarides TJ,
Senagore AJ, Whitlow CB, editors. The ASCRS textbook of colon and rectal surgery. 3rd ed. NewYork:
Springer publishing; 2016.
Lysy J, Sistiery-Ittah M, Israelit Y, Shmueli A,
Strauss- Liviatan N, Mindrul V, Keret D, Goldin
E. Topical capsaicin—a novel and effective treatment for idiopathic intractable pruritus ani: a randomised, placebo controlled, crossover study. Gut.
2003;52(9):1323–6.
Mentes BB, Akin M, Leventoglu S, Gultekin FA, Oguz
M.Intradermal methylene blue injection for the treat-
ment of intractable idiopathic pruritus ani: results of
30 cases. Tech Coloproctol. 2004;8(1):11–4.
Samalavicius NE, Poskus T, Gupta RK, Lunevicius R.Long-
term results of single intradermal 1% methylene blue
injection for intractable idiopathic pruritus ani: a prospective study. Tech Coloproctol. 2012;16(4):295–9.
Smith LE.Perianal dermatologic conditions. In: Gordon
PH, Nivatvong S, editors. Principles and practices
of surgery for the colon, rectum, and anus. 3rd ed.
London: Informa Healthcare publishing; 2007.
Sutherland AD, Faragher IG, Frizelle FA. Intradermal
injection of methylene blue for the treatment of refractory pruritus ani. Color Dis. 2009;11(3):282–7.
Suys E.Randomized study of topical tacrolimus ointment
as possible treatment for resistant idiopathic pruritus
ani. J Am Acad Dermatol. 2012;66(2):327–8.
Ucak H, Demir B, Cicek D, Dertlioglu SB, Akkurt ZM,
Ucmak D, Halisdemir N. Efcacy of topical tacrolimus for the treatment of persistent pruritus ani in
patients with atopic dermatitis. J Dermatolog Treat.
2013;24(6):454–7.
Anal Conditions: Hidradenitis Suppurativa
H.HandeAydinli andEmreGorgun
21
Refer toAlgorithm in Fig.21.1
A. Hidradenitis suppurativa (HS) is a chronic,
progressive inammatory disease of the apocrine gland-bearing skin that most commonly
effects axillary (Fig.21.2b) and perianal areas
(Fig. 21.2d). Overall, ~30% to 50% of
patients with HS have perianal lesions
although the inguinal (Fig.21.2c) and inframammary (Fig. 21.2a) regions can be
involved as well. Perianal HS commonly
appears as a single lesion or combined with
bilateral axillary lesions. Early signs include
open comedones (clogged hair follicle) and
tender subcutaneous papules (Fig. 21.2a).
Additional inammatory nodules can subsequently form and progress to painful draining
abscesses, sinus tracts (Fig.21.2d) and scarring. The skin lesions can interfere with activities of daily living and be difcult to heal.
Patients with severe disease commonly suffer
from poor quality of life and sexual
dysfunction.
The worldwide prevalence is 0.1 to 4%
and females are more likely to be affected.
The mean age of onset is 20 to 24years, and
advanced age is correlated with disease severity. Cigarette smoking and obesity are known
H. H. Aydinli · E. Gorgun (*)
Department ofColorectal Surgery, Cleveland Clinic,
Cleveland, OH, USA
e-mail: gorgune@ccf.org
risk factors. Dietary triggers include dairy
products and highly rened simple carbohydrates. The pathophysiology of the disease
still remains controversial. The most accepted
theory is that follicular epithelial hyperplasia
and infundibular hyperkeratosis lead to follicular occlusion, which subsequently causes
secondary inammation of the apocrine
glands. Endocrine and genetic factors have
been proposed as well.
B. Diagnosing the disease can be challenging
due to the absence of a pathognomonic test.
The reported median delay between the
appearance of initial symptoms and diagnosis
changes from 2.3±5years (mean ± SD) to
12years. In some cases, non-specic lesions
can be confused for other skin conditions
such as simple infections or anogenital
Crohn’s disease, especially if patients seek
care from a number of providers and care
points, including general care practitioners
and emergency rooms. Also, not all patients
present for care, initially. Diagnosis is made
clinically based on the presence of typical
lesions, the distribution patterns of these
lesions and a history of recurrent disease.
Physical examination is the most important
part of the evaluation. Findings generally
include skin thickening, induration, abscess
formation, draining sinuses and contractures.
There are no diagnostic imaging or laboratory
studies specic for HS.Ultrasonography can
© Springer Nature Switzerland AG 2020
S. R. Steele etal. (eds.), Clinical Decision Making in Colorectal Surgery,
https://doi.org/10.1007/978-3-319-65942-8_21
169
170
Fig. 21.1 Algorithm for
the evaluation and
management of
Hidradenitis Suppurativa
(HS)
H. H. Aydinli and E. Gorgun
be used to evaluate skin lesions typically stulous tracts and brotic scarring in lesions,
which might be worse prognostic factors in
terms of response to the medical treatment.
With perianal lesions, the extent of the disease might not always appreciated with physical examination; magnetic resonance
imaging (MRI) may therefore be necessary.
Pelvic MRI may show the extent of the perianal disease and helps exclude Crohn’s disease by revealing anorectal stulizing disease.
In patients with perianal HS, MRI typically
shows subcutaneous edema with possible
supercial sinus tracts; the anal sphincter and
levator plate are usually not involved. Patients
with severe disease should be evaluated for
sepsis with basic laboratory assessment
including a complete blood cell count with
differential, and basic metabolic prole with
C-reactive protein. Biopsy and culture may
be benecial in certain instances of refractory
or atypical disease. According to the stage of
the disease process, typical pathology shows
hyperkeratosis and occlusion of hair follicles,
peri-folliculitis, and invasion of the dermis by
inammatory cells, granulation tissue and
giant cells. It is important to use biopsies
exclude SCC in chronic cases.
C. A number of diseases should be considered in
the differential diagnosis including; acne,
actinomycosis, anal stula, carbuncles, cat
scratch disease, cellulitis, Crohn’s disease,
dermoid cyst, granuloma inguinale, erysipelas, furuncules, inamed epidermoid cyst,
lymphadenopathy, lymphogranuloma venereum, perirectal abscess, pilonidal disease,
and tuberculosis abscess.
D. Different classication/scoring systems have
been created to assess disease severity. The
Hurley classication system is the most commonly used due to its simplicity (Table 21.1).
For a more detailed categorization and/or
research purposes, the Sartorius system and
latent classication systems were established
over time (Tables 21.2 and 21.3). The
Hidradenitis Suppurativa Clinical Response
cd
21 Anal Conditions: Hidradenitis Suppurativa
a b
171
Fig. 21.2 (a) HS inframammary region comedones and
inammation, (b) Left axillary region abscesses, sinus
tracts and scarring, (c) inguinal and suprapubic region
(HiSCR) is a newer and well-accepted
method of assessing the clinical response to
medical treatment in patients with HS. The
HiSCR typically assesses three lesions
including abscesses (uctuant, with or without drainage, tender or painful), inammatory nodules (tender, erythematous, pyogenic
granuloma lesion) and draining stulas (sinus
tracts, with communications to skin surface,
lesions with extensive inammation, (d) perianal region
with abscesses and sinus tracts with seton placement
draining purulent uid). A clinically meaningful response is dened as a 50% reduction
in inammatory lesion count (abscesses and
inammatory nodules) and no increase in the
number of abscesses and draining stulas
when compared from baseline.
E. Several comorbid disorders are correlated
with HS including inammatory bowel diseases (IBD)—mainly Crohn’s disease—