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348 The ASCRS Manual of Colon and Rectal Surgery
• Molluscum contagiosum produces characteristic lesions, papular, 2- to 5-mm diameter, with central umbilication, usually clustered.
• Human immunodefi ciency virus-associated lesions are rarely associated with chronic itching except for secondary fungal infections.
• No credible evidence exists for a viral etiology in idiopathic pruritus ani.
Fungal Infection
• Smith et al. found no instances of fungal infection in their investigation of pruritus in which each of 75 patients had scrapings and fungus cultures.
• Prolonged courses of steroids are said to enhance pathogenicity of C. albicans and to mask Candida infection.
Bacterial Infection
• Several non-sexually transmitted bacterial infections such as beta hemolytic Streptococci and Staphylococcus aureus are reported to cause longstanding pruritus.
• Erythrasma was reported to cause pruritus in 15 of 81 patients (18%) who had failed to respond to routine treatment. Wood’s light fl uorescence (coral pink) was the most reliable diagnostic maneuver, being positive in every case, but cultures of Coryne- bacterium minutissimum were positive in only four cases. Groin, thighs, and toes were also involved in every case and cure was achieved in all patients with erythromycin.
Table 16. 3. Common sensitizing agents. Ethylenediaminetetraacetic acid
Formalin Lanolin (wood wax alcohol) Mercury [Hg(NH Neomycin Nickel Paraben mixtures Paraphenylenediamine Potassium dichromate Rubber ingredients Topical anesthetics (benzocaine, dibucaine) Turpentine oil
)Cl, thimerosal]
2
16. Perianal Dermatology and Pruritus Ani 349
Contact Dermatitis
• Contact dermatitis has been reported from a wide variety of preparations including topical anesthetics, topical antibiotics, topical antiseptics, topical antihistamines, and nickel. Common sensitizing agents identifi ed in the dermatologic literature are listed in Table 16.3 .
• The role of feces and seepage as a contact agent has been emphasized in almost every article devoted to pruritus ani.
• Contact dermatitis may have an irritant or allergic basis, but is recognized by being an eczematous infl ammation characterized by erythema, scale, and vesicles.
• Avoidance of contact with the inciting agent is the obvious treatment, and topical steroids may be useful unless secondary infection is present.
• It is preferable to avoid soaps. Bath oils and emollient creams may be useful for cleansing.
• A large study of patch testing in 80 patients with pruritus ani in Sheffi eld, England, emphasized the importance of contact dermatitis as an aggravating factor. Fifty-fi ve patients tested positive. Thirty-eight of the positives were to medicaments or their constituents including neomycin, fragrance mix, Peru balsam, and cinchocaine. After counseling, two-thirds of these 55 patients experienced improvement or resolution of their symptoms. These authors disputed the recommendation to use “wet wipes” for cleansing because of possible sensitization.
• Bruynzeel corroborates the potential sensitization from use of moist wipes containing methyldibromoglutaronitrile.
• Rohde believes that excessive exposure to water and the act of excessive cleansing itself may incite symptoms, and recom­mends the use of oils for cleaning.
Psoriasis
• Psoriasis has been an important underlying cause of pruritus in every series on this subject.
• Lochridge claimed the diagnosis of perianal psoriasis in 81 patients, all of whom responded to fl uocinolone acetonide
0.025% (Synalar ® ) with normalization of the skin. He recom­mended a search for lesions elsewhere including elbows, knees, ankles, extensor surfaces of the forearm, base of the scalp, ear canals, eyelids, nipples, penis, vulva, or navel.
350 The ASCRS Manual of Colon and Rectal Surgery
Lichen Sclerosis
• Lichen sclerosis (formerly lichen sclerosus et atrophicus) (LS) is a chronic disease of unknown cause, almost always occurring in women (female/male 10:1, usually seen on the penis in the male) which in females has a predilection for the vulva and peri­anal area.
• The skin has a characteristic appearance that is white, atrophic, and wrinkled.
• Involvement of the labia gives this condition a characteristic distribution that makes recognition easy once the diagnosis is considered.
• Treatment of LS with a potent topical steroid (clobetasol pro­pionate 0.05%, Temovate often resulting in normalization of the skin.
• Patients with LS in the vulva probably have a 4–5% incidence of squamous cell carcinoma arising in or adjacent to the LS.
®
) for 6–8 weeks is highly successful,
Food Factors
• No controlled trials have been done to examine food stuffs or diet as a cause for itching, but strong opinions have garnered a revered place in the literature.
• Friend states that virtually all patients with idiopathic pruritus ani consume enormous quantities of liquids, are almost never constipated, and usually have loose stools.
• Friend states that there are six common foods that unequivo­cally cause idiopathic pruritus: coffee, tea, cola, beer, chocolate, and tomato (ketchup) and that total elimination will result in remission of itching in 2 weeks. After a 2-week elimination period, the food may be reintroduced to determine the threshold above which consumption causes symptoms. Thresholds are typically between 2–3 cups of coffee, 4 cups of tea, and less than 2 cans of beer.
• Smith et al. confi rm the importance of poorly formed stool and coffee which may contribute to seepage and recommend a bulk agent taken at the same time of day to promote regular, complete emptying of stool.
16. Perianal Dermatology and Pruritus Ani 351
Coexisting Anal Disease
• Coexisting surgical anal conditions (hemorrhoids, fi ssure, fi stulas) may of themselves produce itching or aggravate any tendency to itch.
• Pirone et al. believe that correction of hemorrhoids, fi ssure, mucosal prolapse, and spasm can resolve fungal infection and the consequent pruritus.
Psychologic Factors
• Anxiety, stress, and fatigue added to personality, coping skills, and obsessive compulsive disorders probably have a role in the exacerbation of pruritus ani. Because of this, psychiatric drugs may have a role in its management in isolated cases.
• The preponderance of evidence suggests that idiopathic pruritus ani does not have a psychiatric basis except as a form of neurodermatitis. The fact that it responds to simple topical treatment with resolution of physical fi ndings in most cases and is so common argues against an obscure etiology.
Steroid-Induced Itching
• Anogenital itching has been reported after bolus administration of intravenous dexamethasone. More often, itching occurs as a rebound phenomenon after withdrawal of steroids leading to their reinstitution and chronic use because symptoms always exacerbate after withdrawal.
• Steroids should always be viewed as potentially dangerous and should be used to achieve specifi c effects. Potency and dosing should be tapered in a planned manner with the goal of eliminating steroids altogether from a maintenance regimen. If elimination is not possible, alternate day therapy or intermittent therapy once or twice a week is to be preferred.
Skin Trauma
• Trauma can arise from physiologic processes such as diarrhea or frequent stools which may be associated with frequent wiping and maceration.
352 The ASCRS Manual of Colon and Rectal Surgery
• Scratching either consciously or nocturnally while asleep may result in the classic lesion of lichen simplex chronicus.
• In most patients the problem is due either to inadequate cleansing of the anus or to over vigorous attempts to “polish it clean.”
• Most authors agree that contact dermatitis is a contributing cause of perianal irritation and that attempts to discontinue over-the-counter preparations (OTCs), perfumed, or scented products including toilet paper, should be made because of potential sensitizing agents (Table 16.3 ).
• Bland emollients, Acid Mantle ® -based creams, and waterless cleansing agents are reasonable substitutes that may be used with tissue paper or cotton balls for cleansing and left on the skin.
• A dilute white vinegar (1 tablespoon in 8 ounces of water) and Burow’s solution (Domeboro ® ) are effective cleansing agents associated with little adverse reaction. Burow’s solution and acetic acid have been found to be an effective antibacterial in chronic otitis with little toxicity.
Neoplasms
• Perianal Paget’s disease is rare and large series do not exist, but more than half of patients in most series have itching, often for longer than 3 months.
• Perianal Bowen’s disease (intraepithelial squamous cell carci­noma in situ) is also rare, but in a series of 47 patients reviewed at the Cleveland Clinic, 28 (60%) had perianal itching as a presenting complaint.
• AIN is the sequel to human papillomavirus infection (associated with itching) and refers to premalignant change in the area of the dentate line and anal transitional zone.
E. Diagnosis of Perianal Disease
• It is often helpful in the differential diagnosis of anal and perianal disease processes to divide them into the general classifi cations of mass (infl ammatory or neoplastic), rash, or fi ssure (primary or secondary).
• The morphology of a lesion is a starting point for diagnosis, but may not be specifi c, and the same disease may have several different appearances (Table 16.4 ).
16. Perianal Dermatology and Pruritus Ani 353
Table 16. 4. Morphology of perianal skin lesions. Ulcers Papules
Herpes genitalis Venereal warts Syphilis Scabies Trauma Molluscum contagiosum Chancroid Candidiasis Fixed drug eruption Syphilis Lymphogranuloma venereum Tularemia Behçet’s syndrome Malignancy Donovanosis (granuloma inguinale) Candidiasis Histoplasmosis Mycobacterioses Amebiasis Gonorrhea Trichomoniasis
Diffuse erythema Crusts
Candidiasis Herpes genitalis Trauma Scabies Contact dermatitis Fixed drug eruption
Miscellaneous fi ndings
Linear tracks: scabies Reddish fl ecks: crab louse excreta Maculae ceruleae (sky-blue spots): crab lice Nits: crab lice Hypertrophic: donovanosis
History and Physical Examination
• History and physical examination, often overlooked in our technologic arrogance, is still the most basic maneuver for diagnosis of any disease (see Table 16.5 ).
• Inquiry about other skin diseases, allergic conditions such as asthma or urticaria, or sites of involvement may be the fi rst clue to diagno­sis of unrecognized psoriasis or atopic dermatitis. Patients may not relate the itch on their elbow to the itch around their anus.
• Erythrasma usually involves the groin and toes, usually is chronic, and is often associated with hyperpigmentation.
354 The ASCRS Manual of Colon and Rectal Surgery
Table 16. 5. Historical and physical factors aiding diagnosis of anal and perianal disease.
Historical
Other skin conditions, asthma, urticaria Prior treatments/OTC topicals Allergies Chemicals/clothes/laundry Antibiotic use Systemic disease Chronicity
Physical fi ndings
Multiple sites (elbows, groins, intertriginous areas, labia, toe webs) Mass or woody induration Hyperpigmentation Scale Lichenifi cation Ulceration Groin adenopathy Defi ned edge or margin
• Patients frequently do not consider OTC or nonprescription preparations as medicines, but these may modify the appear­ance of a condition or even cause it.
• Knowledge of a patient’s allergies is important not only for avoidance, but may aid in uncovering an unsuspected exposure to an occult ingredient.
• Patients sometimes have had patch testing and allergy consulta­tion, and will not volunteer that information unless specifi cally asked. Patch testing, dermatologic consultation, and withdrawal of medication may be in order.
• Specifi c questions about infections, colds, or diarrheal illnesses treated with pills may be necessary to uncover antibiotic use.
• Patients sometimes will not list prednisone in their list of medications until asked a question pertinent to an illness such as arthritis or asthma or myalgias.
• A condition that has come and gone for years or that has seasonal exacerbation may be a clue to anal fi ssure, but could refl ect dietary changes, type of clothes worn, or laundry practices.
• Physical examination should specifi cally look for other sites of involvement. The groin is a classic intertriginous area that is easily accessible in the prone jackknife or the lateral position and should be the fi rst place one looks to confi rm a suspected yeast or fungus diagnosis.
16. Perianal Dermatology and Pruritus Ani 355
• Effective treatment of a patient with changes in the groin as well as the cleft requires attention to each area of involvement.
• A sharply defi ned border usually points to a defi nable diagnosis such as tinea, especially when accompanied by scale.
• Psoriasis usually has a sharply defi ned border, but in the cleft may lack the classic scale seen in skin that is exposed to air. In the confi ned, occluded area of the cleft, there usually is no scale.
• Neoplastic changes may appear sharply marginated, but margins may be microscopically involved, especially around the dentate line, even if grossly normal.
• Infi ltrative processes may be less well defi ned as in Paget’s disease of the anus with the same caveat about margins.
• Infl ammatory changes of idiopathic nature often have borders that are indistinct and nondescript.
• Bright red erythema often is seen with perianal yeast. Erythema may be seen with chronic steroid use.
• Acute severe injury from prolonged diarrhea with frequent wiping produced the picture of lichen simplex chronicus, which was treated by specifi c treatment of the patient’s diarrhea, cleansing with Burow’s solution, and topical silver sulfadiazine to which cortisone was added.
• Chronic infected discharge may lead to hyperpigmentation in the cleft in this case caused by chronic pilonidal disease, but may also occur with fi stulas, chronic yeast or fungus infection, or hidradenitis.
• Severe symptoms, especially paresthesias, coupled with scattered lesions may be a clue to herpes virus infection.
• LS characteristically involves the perineum and labia in the female and has a distinctive appearance with wrinkling of the skin. Biopsy is characteristic.
• Groin adenopathy, and whether or not the nodes are tender, can have specifi c relevance to diagnosis of perianal and anal disease (Table 16.6 ), especially sexually transmitted disease.
Laboratory Examination
• Ideally, infected material should be aspirated with a syringe and expelled into a sterile container. Next best is a swab of exudate collected from a deep portion of the lesion.
• Bacterial and fungal cultures should be placed into a bacterial transport medium and refrigerated if any delay in transport to the laboratory occurs.
356 The ASCRS Manual of Colon and Rectal Surgery
Table 16. 6. Differential diagnosis of groin adenopathy. Benign reactive (shoeless walking)
Lymphoma Carcinoma (penis,vulva, anal canal) Sarcoidosis Syphilis (nontender) Leishmaniasis Chancroid (tender) Herpes genitalis (tender) Lymphogranuloma venereum
• Anaerobic specimens require transport in a special anaerobic medium, and should not be refrigerated.
• The offi ce should have arrangements with a laboratory, which will supply culture swabs with transport media appropriate for aerobic, anaerobic, fungal, and viral culture. These become outdated and can result in rejection of specimens for processing.
• Because staph and strep have been documented as causal agents, it is prudent to culture for pathogens in almost all cases in which treatment is not obvious.
• EMLA ® cream, applied as a lubricant at the time of examination, may facilitate injection of local anesthetic, and biopsy may conveniently be done with either an 11 blade or skin punch blades that come in numerous sizes in separate sterile packages (Fig. 16.1 ). Bleeding from punch biopsy holes is readily controlled with sliver nitrate sticks or GELFOAM ® packing.
F. Treatment of Pruritus Ani
• A general strategy is presented in Table 16.7 .
• Many investigators have alluded to the importance of controlling seepage and fecal contamination of the skin.
• Diet may directly contribute to itching and it is prudent to give patients a list of potential foods implicated in itching for an elimination trial.
• Patients with loose stools may benefi t from the addition of fi ber to absorb moisture and add bulk and improve emptying with defecation.
16. Perianal Dermatology and Pruritus Ani 357
Fig. 16.1. Skin punch biopsy tools come in various sizes up to 1 cm in diameter (2, 3, and 5 mm pictured). They may be purchased as autoclavable sets which may be sterilized and reused, or for the occasional user, disposable punches are supplied in individually wrapped sterile packages. One advantage of the disposable instruments is that they are always sharp.
Table 16. 7. Treatment of pruritus ani.
1. Specifi c directed treatment for a diagnosis
2. Eliminate offending agent [contact irritant (perfume, soap, toilet paper), organism]
3. Eliminate scratching (especially nocturnal)
4. Control symptoms
5. Hygienic measures (Dove
®
soap, detachable shower head, hair dryer to dry)
6. Withdraw inappropriate steroids
7. Treat infection (silver sulfadiazine cream, gentamicin or clindamycin topically, nystatin, clotrimazole)
8. Protect skin [barrier creams, powders (especially athlete’s foot powder)]
9. Correct anal disease (fi ssure, hemorrhoids)
10. Judicious use of appropriate steroids
11. Emphasize control as a chronic condition
12. Reassess diagnosis if response to treatment is not appropriate
13. Anal tattooing in extreme cases