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164
I. Hassan and P.A. Rather
12.3.7 Lichen Simplex Chronicus
Lichen simplex chronicus in the perianal area
appears as an area of lichenifi cation usually unilateral, and localized to the edge of the anus in
one site, and results from chronic, continuous
scratching.
12.3.8 Lichen Sclerosus et
Atrophicus
Lichen sclerosus is a chronic infl ammatory disease that preferentially affects the anogenital
region. Lichen sclerosus in the perianal area has
very rarely been reported in males. In females,
perianal area may be involved, along with vulva,
with thin, wrinkled, atrophic skin, in a characteristic fi gure-of-eight distribution (Wallace 1971 ).
Patients may present with pruritus, soreness/pain,
dyspareunia, urinary or bowel symptoms, or
asymptomatic. Lichen sclerosus is a scarring disease and some architectural change is common.
The diagnosis is established by a combination of
the characteristic clinical and histological fi ndings. Complications include scarring and malignant transformation.
12.3.9 Hidradenitis Suppurativa
Hidradenitis suppurativa is a disease of the skin
containing apocrine glands, especially the axillae,
groin, and buttocks, along with perianal area. Pore
occlusion of the apocrine glands leads to stasis,
bacterial infection, and the resultant formation of
characteristic tender, erythematous, hard nodules
that may evolve into fl uctuant interconnecting
abscesses, which rupture leading to sinus tract
formation, and multiple fi stulous tracts.
Hidradenitis suppurativa may result in varying
degrees of infl ammation and scarring (Fig.
It is more common in black and Mediterranean
individuals. In established hidradenitis, a variety
of presentations may be found such as bridged
comedones, folliculitis and furunculosis, deep
burrowing discharging sinuses, nodules, cysts,
12.2 ).
Fig. 12.2 Hidradenitis suppurativa
fl uctuant abscesses, scarring, and fi brosis (Coda
and Ferri
1991 ). Urethral–cutaneous fi stula and
phimosis may occur (Chaikin et al. 1994 ).
Severe degrees of morbidity occur with
interference with sitting, sleeping, walking,
defecation, and sexual activity, which may lead
to depression. Long-duration disease carries a
signifi cant risk of progression to squamous cell
carcinoma (SCC) and rarely verrucous carcinoma (Black and Woods 1982 ; Cosman et al.
2000 ). Hidradenitis is usually a clinical diagno-
sis. Investigations include taking swabs for bacteriological evaluation and to guide therapy,
evaluation for sexually transmitted diseases,
and skin biopsy to exclude carcinoma or
Crohn’s disease.
Treatment is challenging. Phenolization of
small localized lesions and intralesional corticosteroids for early lesions may help.
Marsupialization, diathermy destruction of the
affected tissue, and carbon dioxide laser have
been found to be effective (Brown et al.
1986 ;
Finley and Ratz 1996 ). Silastic foam dressing
may facilitate healing. Plastic surgery with complete excision of all the involved skin may be
required. Medical management in the form of
long-term antibiotic therapy (erythromycin, fl ucloxacillin, ciprofl oxacin, metronidazole), oral
prednisolone, and isotretinoin (1 mg/kg) for 6–8
months has proven helpful (Highet et al. 1988 ;
Brown et al. 1988 ). Antiandrogen therapy and
biologics such as infl iximab and other TNF “biologicals” are being evaluated (Revoz 2009 ).
12 Peria nal Der matol o g y
165
12.3.10 Crohn’s Disease (Synonym: Regional Ileitis)
Crohn’s disease is an infl ammatory granulomatous disease of the gastrointestinal tract. Crohn’s
disease can affect any part of the gut and perianal
disease may occur in up to 75–90 % of patients
(Markowitz et al. 1984 ). Perianal area may be
involved by Crohn’s disease either by metastatic
spread or by direct extension into the perianal
region. Clinical features include those common
to most chronic diarrheal illnesses, such as pruritus ani, skin maceration, and erosions with secondary infection.
Deep, undermined, angulated fi ssures with cyanotic edges and less commonly fi stulae are common features, with multiple external openings
encountered all over the perianal area and buttocks, scrotum, and thighs. Relative lack of pain,
multiplicity of lesions, and eccentricity of fi ssures
are important pointers for the diagnosis of Crohn’s
disease (Alexander-Williams and Buchmann
1980 ). Any anal lesion in a patient who is known
to be suffering from Crohn’s disease is likely to be
perianal Crohn’s, and diffi culty arises when anogenital disease represents the fi rst manifestation.
Diagnosis may be achieved based on symptoms,
signs, and investigation results (e.g., radiography
and biopsy) consistent with Crohn’s disease. Anal
stenosis, fecal incontinence, and carcinoma are
complications (Slater et al. 1984 ).
The differential diagnosis includes nonspecifi c anal fi ssures and fi stulae, lesions of ulcerative colitis and diverticulitis (much less
common), hidradenitis suppurativa, proctitis,
perianal ulceration, abscess, fi ssure and fi stula in
homosexual men and those with HIV/AIDS, and
pyoderma gangrenosum (Denis et al.
Other differential diagnoses include sarcoidosis,
schistosomiasis, leishmaniasis, tuberculosis,
atypical mycobacterial infection, deep fungal
infection, granuloma inguinale, lymphogranuloma venereum, chancroid, amoebiasis, syphilis
and rarely condylomata acuminata, anorectal carcinoma, and other mucocutaneous malignancies
(basal cell carcinoma, Kaposi’s sarcoma, and
amelanotic malignant melanoma).
1992 ).
Fig. 12.3 Perianal involvement in acrodermatitis
enteropathica
Management includes treatment of the underlying intestinal Crohn’s disease and local measures including soaks with potassium
permanganate and aluminum acetate, potent or
very potent topical corticosteroid/antibiotic combinations, and oral antibiotics (as for hidradenitis). A role has been advocated for long-term oral
metronidazole (20 mg/kg/day in divided doses),
sulfasalazine, prednisolone, and azathioprine
(Bernstein et al.
1980 ; van Assche et al. 2009 ).
12.3.11 Miscellaneous Infl ammatory
Dermatoses
Acrodermatitis enteropathica presents with perianal eczematous dermatitis in the perianal area
(Ecker and Schroeter
defi ciency diseases with some similarity to acrodermatitis enteropathica are pellagra, maple
syrup urine disease, and neonatal citrullinemia.
Radiodermatitis may result following previous treatment for in situ or frank carcinoma or
pruritus ani.
Lichen planus (LP) involving perianal region
may become excoriated or hypertrophic.
Fixed drug eruption and Stevens–Johnson
syndrome may produce anal and perianal
lesions.
Bullous disorders such as cicatricial pemphigoid, Hailey-Hailey disease, and epidermolysis
1978 ) (Fig. 12.3 ). Other
166
I. Hassan and P.A. Rather
Fig. 12.5 Fistula-in-ano
causing pressure trauma and necrosis, or postoperative complications or idiopathic. Symptoms
include intense pruritus, pain, bleeding, mucous
discharge, and constipation. There may be a
“sentinel pile” at the anal pole of the ulcer.
Management is both medical and surgical.
Fig. 12.4 Perianal involvement in epidermolysis bullosa
bullosa (Fig. 12.4 ) may affect perianal skin and
may cause anal stenosis.
Behçet’s disease occasionally presents with
multiple shallow ulcers and fi ssures of the anal
margin. Behçet’s disease is a rare multisystem
infl ammatory disorder in which recurrent oral
aphthae combine with some of the following
clinical features: genital erosions or ulcers, arthritis, uveitis, neurologic disorders such as cranial
nerve palsies and mono- and hemiparesis, arterial
and venous thromboses, and pathergy.
Calciphylaxis sometimes affects the thighs
and buttocks.
Primary systemic cutaneous anosacral amyloidosis has a predilection for the anogenital region,
particularly the sacrum (Mukai et al.
1986 ).
12.3.12 Other Related Infl ammatory
Dermatoses
12.3.12.1 Anal Fissures
A true anal fi ssure is a midline linear perianal
ulcer that is caused by defecation of hard stools
12.3.12.2 Anal Fistula
Communication between the anal canal and the
perianal skin is mostly found in the midline posteriorly, though there may be multiple openings
(Fig. 12.5 ). Fistula may arise from infection/
abscesses within the anal glands, Crohn’s disease,
foreign body, and tuberculosis, to mention a few.
Pruritus ani related to seropurulent discharge is the
usual presentation along with pain resulting from
abscess formation. Management is mainly surgical.
12.3.12.3 Pilonidal Cyst/Sinus
Perineal pilosebaceous unit and precursor pits
associated with trapped hairs result in formation
of pilonidal cyst/sinus (Millar 1970 ). Pilonidal
sinus occurs in the midline, sacrococcygeal
location being the most common site. It may
present as a nodule or cyst, which ruptures and
becomes infected. Symptoms include itching,
pain, recurrent abscess, purulent discharge, and
persistent nodule. Clinically, pilonidal sinus constitutes part of the “follicular–occlusion tetrad,”
along with hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp.
Treatment is symptomatic and mainly surgical
(Allen-Mersh 1990 ).
12 Peria nal Der matol o g y
Fig. 12.6 Flowchart showing
pathogenesis of pruritus ani
167
Difficulty in
cleansing the area
Anal leakage
Pruritus ani
12.3.12.4 Pruritus Ani
Pruritus ani in itself is not a diagnosis, but a
symptom complex having many causes; almost
50 % have a cause after dermatological evaluation (Jones 1992 ). It may be associated with vari-
ous forms of anal diseases and with skin
conditions involving the perianal area. Anal itching occurs in association with any infl ammatory
or eczematous condition of the perianal skin, anal
fi ssures, anal fi stulae, piles, skin tags, malignant
tumors, mycotic infections, candidal infections,
threadworm infestation, staphylococcal infection, folliculitis, erythrasma, warts, underlying
skin diseases such as psoriasis, atopic dermatitis,
lichen planus, lichen sclerosus, systemic disease
pellagra, hypovitaminoses A and D, diabetes
mellitus, and psychological and idiopathic factors (Harrington et al.
1992 ).
Various clinical features result secondary to
the effects of rubbing, secondary infection, and
contact dermatitis.
Common causative factor of pruritus ani is
fecal contamination (Kocsard 1981 ) (Fig. 12.6 ),
because of irritant potential and presence of
potential allergens and endopeptidases of bacterial origin, capable of inducing itching in the
presence of preexisting skin disease (e.g., seborrheic dermatitis or fl exural psoriasis) or even in
the absence of visible disease (Caplan 1966 ;
Andersen et al. 1994 ). Anal leakage may result
from coexisting anal disease, exaggerated
Fecal
contamination
Bacterial
contamination
Food and drink
recto- anal inhibitory refl ex, or anal sphincter
dysfunction (Allan et al.
1987 ; Eyers and
Thompson 1979 ) or be precipitated by broad-
spectrum antibiotics and diarrhea.
The causes of fecal contamination include:
(a) Diffi culty in cleansing the area because of
obesity, frequent defecation, and anatomical
factors
(b) Anal leakage because of hemorrhoids, peri-
anal tags or fi ssures, and primary anal sphinc-
ter dysfunction.
(c) Bacterial contamination
(d) Food and drink – uncertain, although com-
pelling evidence
General measures include attention to the
patient’s washing habits, suitable soap substitute;
moisturizer after each wash; barrier preparation
pre-applied to the perianal skin before the bowel
opening; washing preferred to wiping with toilet
paper; wearing loose cotton underwear; avoiding
topical anesthetics; curtailing coffee consumption; excluding foods, such as nuts, that provoke
the pruritus; and encouraging high-fi ber diet
(Alexander-Williams
1983 ). Local applications
of mild topical corticosteroid/antibiotic/antifungal preparation are useful for acute episodes.
Other treatments that have been advocated
include zinc paste with 1–2 % phenol, halfstrength Castellani’s paint, weak (0.05–0.25 %)
168
I. Hassan and P.A. Rather
silver nitrate solution, oral antihistamines,
corticosteroid suppositories, systemic corticosteroids, and intralesional methylene blue, with or
without Marcaine/epinephrine/xylocaine
(Eusebio 1991 ). Concomitant diseases such as
hemorrhoids, fi ssures, anal spasm, and occult
mucosal prolapse should be treated. Lord’s
stretch procedure has proved helpful (Ortiza et al.
1978 ) and above all reassurance.
shows a pronounced, sharply demarcated, and
boggy erythema and most commonly affects
children younger than 10 years of age (Rehder
et al. 1988 ). Rarely, there may be a systemic pre-
sentation with fever and rash (Vélez and Moreno
1999 ). Group A β-hemolytic streptococci is the
usual cause, and rarely S. aureus, and communal
bathing has been blamed for outbreaks. Diagnosis
rests on clinical presentation, response to medication, and identifi cation of bacteria through culturing of the lesion.
12.4 Infections
12.4.1 Folliculitis and Furunculosis
The perianal area is susceptible to infection with
Staphylococcus aureus , involvement commonly
being in the form of furunculosis and abscesses
(Fig. 12.7 ). High temperature, humidity, pres-
sure, and friction encourage colonization by
staphylococci (Felman and Kikitas 1980 ). The
perineal area is an important site for carriage of
staphylococci. In adults, the carriage rate is of the
order of 13–22 %, and in neonates, it may be
higher.
12.4.2 Streptococcal Dermatitis/ Perianal Cellulitis
Superfi cial bacterial infections of the perianal
area present with pruritus, painful defecation,
anal soreness and redness, and satellite pustulosis
of the buttocks, and examination of the anus
12.4.3 Perianal Abscess
Perianal/anorectal/ischiorectal abscess presents
with painful swelling and suppuration, commonly complicated by anal fi stula. The most
likely cause is infection of the anal glands, but
trauma (e.g., impacted fi sh bone), diabetes, and
anal cancer predispose to its development.
12.4.4 Ecthyma Gangrenosum
Ecthyma gangrenosum in the perianal area,
caused by gram-negative organisms (pseudomonas aeruginosa), occurs if the balance of
the skin flora is grossly disturbed. Patients
present with severe anal pain, anorectal ulceration, and septicemia, and the prognosis is
poor (Givler 1969 ).
12.4.5 Necrotizing Infections
Perianal area may be affected by a number of
severe gangrenous and necrotizing diseases,
often as a complication of surgery and trauma.
These conditions include clostridial and
non- clostridial gangrene; streptococcal cellulitis
and myositis; streptococcal toxic shock syndrome; synergistic necrotizing cellulitis; necrotizing fasciitis; Meleney’s progressive bacterial
synergistic gangrene; synergistic gangrene; and
Fournier’s gangrene (Bubrick and Hitchcock
1979 ; Oh et al. 1982 ; Flanigan et al. 1978 ). Fig. 12.7 Furuncle in perianal area
12 Peria nal Der matol o g y
ab
Fig. 12.8 Necrotizing fasciitis in the perianal area
169
The condition may present as a primary perirectal abscess in the perineum, with pain generally being the fi rst symptom. Subsequently,
distinct dusky red erythema and necrotized area
may appear in affected tissue, with tenderness
and extension to wider areas leading to fasciitis
and myositis (Fig. 12.8 ). Crepitus is an important
feature, as is the presence of a dark brown, turbid
fl uid without pus. Bad prognostic factors are
patients with diabetes, leukemia, old age, and
delay in treatment.
Early recognition along with immediate and
aggressive treatment is essential. High-dosage
broad-spectrum antibiotic therapy should be
started till results of the culture and sensitivity
are available. Rapid and extensive debridement
of all affected tissue may be needed.
12.4.6 Common Mycoses
The yeast, candida albicans, causes candidal
intertrigo, found between the gluteal folds and
also perianal dermatitis, often precipitated by use
of oral antibiotic agents, steroid use, and pregnancy. Perianal candidiasis presents with pruritus
ani and a more localized erythema, around the
anus. There is a bright red, glazed appearance,
often with outlying small pustules. Diagnosis
relies upon clinical fi ndings and microscopic
examination of scrapings with potassium hydroxide for hyphae/pseudo-hyphae.
Tinea is dermatological infection caused by
dermatophyte fungi, with a predilection for moist
environments, including perianal area, and Tinea
cruris may spread back around to the anus.
Clinical features include pruritic, erythematous
patches with elevated borders that are serpiginous with scaling. The borders can have papules,
pustules, and vesicles. Diagnosis may be supplemented by scraping of the scale and microscopic
evaluation with potassium hydroxide (KOH)
preparation which reveals diagnostic hyphae and
by fungal culture. The possibility of fungal infection should be considered in all unusual forms of
perianal dermatitis, as the typical lesions of fungal infection are usually modifi ed by corticosteroid application (Fig.
12.9 ).
Erythrasma is a relatively rare skin infection
caused by Corynebacterium minutissimum that
affects mainly the intertriginous skin and may
also involve perianal area. It presents as asymptomatic light brown patch with sharp borders of
irregular contour, showing a diagnostic coral
red- colored fl uorescence under Wood’s lamp
(Bowyer and McColl
1971 ).
12.4.7 Thread/Pinworms
Thread/pinworms can cause pruritus ani, excoriations, eczematization, impetiginization,
and rarely perianal abscess (Mortensen and
Thomson 1984 ).
170
I. Hassan and P.A. Rather
Fig. 12.9 Tinea infection modifi ed by application of
corticosteroids
12.4.8 Sexually Transmitted Diseases (STDs)
Syphilis may present in different ways in the
perianal area, and it should never be forgotten as
a possible cause of anal ulceration. Anal chancres
are often mistaken for fi ssures and fi stulae.
Bilateral lymphadenopathy is extremely rare
with other perianal ulcers. Painful syphilitic
proctitis in the absence of anal lesions can occur
(Akdamar et al. 1977 ). Moist, fl at condylomata
lata and granulomatous gumma may affect the
anal area as an ulcer, a white plaque, or an atrophic scar.
Herpes simplex infection, primary or recurrent, may involve perianal area, sometimes
exclusively.
Chancroid may cause extremely painful anal
lesions instead of the classic multiple soft
chancres.
In granuloma inguinale, the initial rapidly
ulcerating papule may occur in the perianal
region in homosexual males, as soft, painless
lesion which bleeds easily on trauma. “Pseudobubo” may be present. In the anal canal, the
lesion never extends beyond the stratifi ed epithelium and strictures do not occur, but anal
stenosis or, rarely, epitheliomatous changes can
supervene.
Lymphogranuloma venereum causes ulcerative proctitis and widespread vegetating and
scarring lesions (Collins et al.
and complement fi xation tests distinguish it from
hidradenitis suppurativa.
2006 ). The Frei
Gonorrhea can result in anal infl ammation and
discharge or edematous perianal dermatitis with
multiple fi ssures and erosions.
Perianal viral warts (condylomata acuminata)
common in young adults are not always sexually
transmitted. They may be extraordinarily profuse, extending into the anal canal, especially in
homosexuals or in immunocompromised subjects, with a higher risk of progression to dysplasia and frank malignancy (Daneshpouy et al.
2001 ) (Fig. 12.10 ). Biopsy should be performed
if there is diagnostic doubt or if dysplasia is suspected. Patients with perianal warts, and their
partners, may require full STD and sometimes
colorectal assessment. HPV infection in the perianal area can be very diffi cult to treat.
Molluscum contagiosum may also involve the
perianal area, with characteristic pearly, waxy
umblicated papules, sometimes giant lesions.
Human immunodefi ciency virus (HIV) infection: Perianal ulceration may occur in homosexual men with HIV/AIDS. The main causes of
anal ulceration in HIV infected patients are hemorrhoids, fi ssures, sepsis (abscess, fi stula), syphilis (chancre), herpes simplex, cytomegalovirus
(CMV), Epstein–Barr virus infection, gonorrhea,
and anal warts (Berger et al. 1988 ).
Other causes include amoebiasis, Kaposi’s
sarcoma due to ano-receptive anal intercourse
and human herpes virus (HHV) 8 (Lorenz et al.
1990 ), non-Hodgkin’s lymphoma, and squamous
cell carcinoma and are idiopathic. Biopsy with
special stains and culture is mandatory.
12.4.9 Miscellaneous Infections
Perianal tuberculosis is seen where tuberculosis
is common, especially developing countries. A
primary lesion is exceptional and accompanying
unilateral lymphadenopathy is an important feature. Primary lesion may present as indolent,
irregular, painful ulcers, fi stulae, and abscesses.
Lupus vulgaris, verrucous tuberculosis, and orifi cial tuberculosis cutis may also occur. Perianal
scrofuloderma (secondary skin involvement from
underlying lymph node disease) may cause diagnostic confusion with pyoderma gangrenosum,
12 Peria nal Der matol o g y
ab
Fig. 12.10 Perianal viral warts (condylomata acuminata)
171
Crohn’s disease, hidradenitis, neoplasia, sexually
transmitted diseases, amoebiasis, and deep mycoses (Betlloch et al. 1994 ).
Sacral herpes zoster is rare and may cause signifi cant morbidity from acute cystitis or urinary
or fecal retention when involving S2–S4 or, less
commonly, the ilioinguinal segment of L1–L2
(Waugh 1974 ).
Amoebiasis of the perianal skin is usually
associated with bowel infections but, where the
disease is endemic, direct inoculation of abraded
skin or operation wounds can occur. Abscesses,
fi stulae, slowly extending ulcers with serpiginous
cord like margins and whitish slough with black,
and foul-smelling eschar may be the presentation. Progression may be rapid, leading to complete destruction of the perianal and sacral tissues
(Wynne
1980 ). The diagnosis is made by fi nding
the Entamoeba species in a biopsy specimen
from the edge of a lesion or by examination of a
fresh sigmoidoscopy swab. Treatment with metronidazole may be dramatically effective, but in
severe cases, surgery may also be required.
Coxsackie infections in infants can cause a
transient papular or papulovesicular eruption of
the perianal area.
Kawasaki disease may show an erythematous,
desquamating, perineal eruption in the fi rst week
of the disease (Friter and Lucky
1988 ). Additional
fi ndings include a strawberry tongue, fi ssured
lips, fever, and lymphadenopathy, followed by
desquamation of the hands and feet.
Daughter yaws, presenting as papules which
rapidly become ulcerated crusted plaques, have a
predilection for periorifi cial sites on the face and
around the perineum.
The rare giant condyloma of Buschke–
Löwenstein is probably human papilloma virus
(HPV)-related (Alexander and Kaminsky
1979 ).
Orfalso occurs occasionally in the perianal
area (Kennedy and Lyell 1984 ).
Perianal cytomegalovirus (CMV) ulceration is
extremely rare in the immunocompetent and
occurs commonly in HIV infection.
Trichosporosis is a common cause of perianal
intertrigo in India, causing itching or burning.
Schistosomiasis (bilharziasis) may rarely
present as perianal granulomatous lesions, presenting as pruritic papules, in countries where it
is endemic (Adeyemi-Doru et al. 1979 ). It is usu-
ally preceded by rectal or intestinal symptoms.
The papules and nodules may be skin-colored,
172
I. Hassan and P.A. Rather
pink or brown, scattered, or grouped as warty
lesions. The anogenital lesions result from ova
shed by worms that have entered the perineal vessels. Viable or calcifi ed ova may be found in the
dermis.
The perianal region is an extremely rare site
for cutaneous leishmaniasis (Aste et al. 1997 ).
Larva currens, caused by Strongyloides ster-
coralis , commonly occurs around the anus, therefore a common cause of excoriation (Ho et al.
1997 ). Cutaneous larva migrans resulting from
the dog hookworm Ancylostoma braziliense may
occur around the pelvic girdle.
Perianal histoplasmosis, blastomycosis, and
actinomycosis have also been recorded (Grigoriu
and Delecretaz 1981 ).
12.5 Benign Tumors
Many benign lesions are encountered in the perianal area. Angiomas and angiokeratomas are
common (Fig. 12.11 ). Others include basal cell
papillomas, melanocytic nevi, inguino-genital
epidermoid cysts, lesions containing molluscum
contagiosum, and pilar cyst (very rarely).
12.5.1 Hemorrhoids
and prolapse. Signs depend on the presentation.
In the perianal area, skin tags from fi brosed piles
are extremely common.
12.6 Premalignant Dermatoses and Frank Malignancies
Solar radiations are the principal cutaneous carcinogen, and fortunately perianal area is not directly
exposed to it. But use of tar and radiotherapy in the
past for pruritus and for gynecological malignancy
carries theoretical hazards and can occasionally be
incriminated in carcinogenesis. Scarring following
chronic granulomatous processes in the area is
important ground and potential hazard for malignancy in the area. Condylomata acuminata have
preceded squamous cell carcinoma (SCC) of the
anal or perianal skin and HPV is a major suspect in
the precancerous process (Sturm et al. 1975 ).
12.6.1 Porokeratosis
Anogenital porokeratosis of Mibelli is rare, but
classic lesions have been found in the natal cleft
and may cause ulceration. It may be confused
with psoriasis, Bowen’s disease, granuloma
annulare, or LP. Histopathology confi rms the
diagnosis (Levell et al. 1994 ).
Clinical features of hemorrhoids/piles include
rectal bleeding, mucous discharge, pruritus ani,
12.6.2 Anal Intraepithelial Neoplasia
Anal intraepithelial neoplasia (AIN) describes fullthickness dysplasia of the anus and perianal skin. It
may present as relatively asymptomatic red, shiny, or
scaly patches like Bowen’s disease or as warty lesions
like bowenoid papulosis (BP). AIN is frequently
associated with homosexuality, anal warts, and
HIV. The risk of AIN progression to carcinoma may
not be as low as earlier thought (Morgan et al. 1994 ).
12.6.3 Carcinoma of the Anus
Anal squamous carcinoma is sometimes called
epidermoid carcinoma. Fifty-six percent of all Fig. 12.11 Perianal and gluteal angiokeratomas
12 Peria nal Der matol o g y
173
anal carcinomas are of the squamous variety
(Boman et al. 1984 ).
Exact etiopathogenesis is not fully elucidated,
but associations with smoking; cervical intraepithelial dysplasia; changing sexual habits, including homosexual anal intercourse; coexistent
Crohn’s disease; and HPV, especially HPV 16,
have been found (Slater et al. 1984 ; Frisch et al.
1993 ; Kadish 2001 ; Chang et al. 1990 ). A role for
seminal fl uid prostaglandins in homosexual anal
cancer has also been proposed (Kondlapoodi
1982 ). Immunosuppression, including by HIV
infection, is a risk factor for AIN and anal cancer.
Clinical features include bleeding, pain, and
change in bowel habits. Examination reveals a
hard mass that may be fl at, raised, or polypoid.
Squamous carcinoma should be suspected in all
nodulo-ulcerative anal and perianal diseases,
especially in the context of lichen sclerosus,
hidradenitis suppurativa, intraepithelial neoplasia, and Immunocompromised subject.
In the management of anal carcinoma, consideration should be given for the preservation of
sphincter function, and this frequently involves
combined radiotherapy and chemotherapy
(Esiashvili et al. 2002 ). Small squamous carcino-
mas may respond well to radiotherapy. Surgical
excision of the tumor, and of the inguinal lymph
nodes when these are involved, is the treatment of
choice. For small, well-differentiated tumors,
particularly adenocarcinomas, local excision and
repair are ideal.
12.6.4 Extra-Mammary Paget’s
Disease (EMPD)
Paget’s disease, most commonly associated with
the nipple, can also be found at a number of extramammary sites, including the perianal region,
where extra-mammary Paget’s disease (EMPD) is
a rare, but important, diagnosis (Butler et al. 1997 ).
Most cases with perianal EMPD present as
pruritus ani (Redondo et al. 1995 ). EMPD can be
primary, arising as an intraepithelial adenocarcinoma, or secondary, due to pagetoid spread of an
adjacent or contiguous in situ or invasive
neoplasm. Thorough search for a primary
adenocarcinoma of underlying secretory glands
should be carried out in perianal EMPD. The primary tumor is an anorectal, or even more distant,
carcinoma (Helwig and Graham
tial presentation is often a bland, sharply demarcated persistent eczematous patch that can be
intensely pruritic and/or painful. Bleeding may
present as a later manifestation.
EMPD may become invasive and metastasize
via the lymphatic system. Diagnosis relies upon
biopsy with histology showing hyperkeratosis,
parakeratosis, acanthosis, and pale Paget’s cells
in the rete ridges. Extensive surgery may be necessary, with micrographic margin control and
plastic repair (Coldiron et al. 1991 ). Photodynamic
therapy and laser treatment have been used
(Petrelli et al. 1992 ).
1963 ). The ini-
12.6.5 Miscellaneous Malignancies
Although basal cell carcinoma is one of the most
common types of skin cancer, it is rare in the anogenital area (Gibson and Ahmed 2001 ). Anorectal
melanoma accounts for only 1 % of all tumors of
this area (Johnson et al. 1993 ). Anogenital Kaposi’s
sarcoma is essentially an HIV-related problem.
Non-Hodgkin’s lymphoma of the perianal
area has been described in HIV/AIDS (Denis
et al. 1992 ).
Perianal infi ltration, ulceration, or abscess
occurs in 5 % of hematological malignancies
(Vanheuverzwyn et al.
Perianal metastases from transitional cell carcinoma of the distal urethra, from rectal carcinoma, and from epidermoid anal canal carcinoma
have occurred. Carcinoma erysipeloides has been
observed in the perineum and on the thigh in carcinoma of the bladder and prostate (Cohen and
Kim 1980 ).
Other tumors include fi brosarcoma, hemangiopericytoma, leiomyosarcoma, malignant
fi brous histiocytoma, epithelioid sarcoma, dermatofi brosarcoma protuberans, and spindle cell
sarcoma and may present as painful or painless
nodules, masses, or swellings. Merkel cell
carcinoma, malignant eccrine poroma, and
malignant schwannoma may also occur.
1980 ).