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Perianal Dermatology/Puritis Ani A Corman Review Justin Blasberg, MD 9/22/05 What to look forward to? Description of skin conditions affecting the perianal area Review of the differential diagnosis Examples of common and uncommon findings Treatment of the relevant diseases Classification of Skin Conditions Inflammatory Infectious Neoplastic Inflammatory Pruritus ani Psoriasis Lichen planus Lichen sclerosus et atrophicus Atrophoderma Contact (allergic) dermatitis Seborrheic dermatitis Radiodermatitis Behcet’s syndrome Lupus erythematosus Dermatomyositis Scleroderma Erythema multiforme Familial benign chronic pemphigus (i.e. Hailey-Hailey) Pemphigus vulgaris Cicatricial pemphigoid Infectious Nonvenereal: Pilonidal sinus Suppurative hidradenitis Anorectal abscess and anal fistula Crohn’s disease TB Actinomycosis Fournier’s gangrene Ecthyma gangrenosum Herpes Zoster Vaccinia Tinea cruris Candidiasis “Deep” Mycoses Ambebiasis cutis Trichomoniasis Schistosomiasis cutis Bilharziasis Oxyuriasis (i.e. pinworm, enterobiasis) Creeping eruption (i.e. larva migrans) Larva currens Cimicosis (i.e. bedbug bites) Pediculosis Scabies Infectious Venereal: Gonorrhea Syphilis Chancroid Granuloma inguinale Lymphogranuloma venereum (Chlamydia infection) Molluscum contagiosum Herpes genitalis Condylomata acuminate Neoplastic Acanthosis nigricans Leukoplakia Mycosis fungoides Leukemia cutis Basal cell carcinoma Squamous cell carcinoma Malignant melanoma Bowen’s disease Extramammary Paget’s disease Pruritus Ani “itching in the anal area” Symptoms: Itching of anal and genital areas Worsening at night May awaken the patient from sleep Scratching with exacerbation of complaint Chronic itching can lead to atrophic or hypertrophic skin, with associated nodularity and scarring Pruritus Ani Differential Hemorrhoids Anal fissure Scarring from prior anal surgery Constipation/diarrhea Contact dermatitis Mycoses Seborrhea Diabetes Pinworm Psoriasis Neurodermatitis Why me, why now? Increased anal sphincter relaxation in response to rectal distension Abnormal rectoanal inhibitory reflexes and a lower threshold for internal sphincter relaxation Evaluation Anoscopy and proctosigmoidoscopy Magnifying lens Woods lamp Skin scrapings Stool assessment? What you might see Marked edema with papillomatosis and nodularing resulting from chronic abrasion Treatment Injections of local anesthetics, phenol, and alcohol Methylene blue Diet modification Sterilization? Antibiotics? Psoriasis Chronic inflammatory disease of the skin Characterized by rounded circumscribed erythematous dry scaling patches covered by grayish white or silvery white scales Predilection for scalp, nails, extensor surfaces or limbs, elbows, knees, and sacral regions Butterfly distribution over the coccyx and sacrum Treatment Moisturizers and agents with salicylic acid Topical corticosteroids Coal tar Anthralin Retinoid Vitamin D3 derivatives Ultraviolet B light PUVA treatment Methotrexate and Cyclosporine Lichen Planus Eruption of small, flat-topped papules with a distinct violaceous color and polypoid configuration Found in flexor surfaces, mucous membranes, genitalia, and perianal area Focal thickening of the granular layer, degeneration of the basement membrane and basal cells, and a bandlike lymphocytic infiltrate in the upper dermis Diagnosis made with skin biopsy Treatment with corticosteroids and occlusive dressings What you might see Moderate hyperkeratosis, thickening of the stratum granulosum, saw tooth configuration of rete ridges, and lymphocytic infiltration Irritant and Contact Dermatitis Irritant: Nonallergic reaction following exposure to an irritating substance Alkalis, acids, metal salts, dusts, gases, and hydrocarbons Allergic (contact): Allergic sensitivity to a number of responsible agents, also known as hypersensitivity of the delayed type (cell mediated hypersensitivity) Dyes, oils, resins, chemicals used on fabrics, cosmetics, insecticides Radiodermatitis Secondary to radiotherapy of the rectum, anus, and prostate Cell mitosis is arrested; skin change results from the dosage of radiotherapy Erythema, edema, ulceration, and symptoms of burning, itching, or severe pain Treatment with oral Vitamin A 8000IU BID Hyperbaric O2 has also been found to be helpful What you might see Fibrosis of the dermis with sclerosis, atrophy of the epidermis, and absence of skin appendages Pilonidal Sinus Common infective process occurring in the natal cleft and sacrococcygeal region Affects young adults and teenagers 3:1 male predominance Epithelium lined sinus is usually found to contain hair Sinus may become infected, usually after puberty, with drains openings overlying the coccyx and sacrum Infected abscess may extend to the perianal area that may be mistaken for an anal fistula Why me, why now? 2 Theories of formation: Failure of fusion in the embryo, with entrapment of hair follicles in the sacrococcygeal region Result of trauma, with the introduction of hair shafts into the subdermal area Symptoms Pain, swelling, purulent drainage at and around the site of the pilonidal opening Typical appearance of an abscess may be evident Fever and leukocytosis may be present What you might see Multiple openings overlying the sacrum and buttocks What you might see Indolent, granulating, nonhealing wound of a recurrent (persistent) pilonidal sinus Treatment Antibiotics? Adjuvant to a surgical procedure I&D Definitive therapy: Excision, excision with grafting or with an open wound to close secondarily, cryosurgery, and injection of sclerosing agents Tuberculosis Confused for Crohn’s, actinomycosis, anal fistula, colloid carcinoma, sarcoidosis, other skin conditions Anal fistula is the most frequent presentation Lesion appears as brownish red papule that can progress to an ulcerating plaque Anal fissure in an unusual location that is slow to heal should raise the suspicion Treatment: anti-TB drugs with resolve usually in 2 to 3 weeks STD’s Gonorrhea Chancroid Chlamydia Herpes Simplex Syphilis: Chancre Condylomata lata What you might see Large perianal mucoid warty mass composed of smooth-surfaced lobules Neoplastic Premalignant Lesions Acanthosis Nigricans-ominous association with abdominal cancer Affects face, neck, axillae, external genitalia, groin, inner thighs, umbilicus, and anus Grayish velvety thickening or roughening of the skin Epidermal papillomatosis, hyperkeratosis, and hyperpigmentation Treatment is directed to the primary malignant condition Premalignant Lesions Leukoplakia Whitish thickening of the mucous membrane epithelium occurring in patches of diverse size and shape Seen in the anal canal Associated with an increased risk of malignancy/epidermoid carcinoma Symptoms of bleeding, discharge, and pruritic symptoms are the most common complaints Hyperkeratosis and squamous metaplasia Skin Cancer Basal Cell Carcinoma Most common cutaneous malignancy, extremely rare in the anal area Tumors usually between 1-2 cm Presents with a lump or ulcer Bleeding, pain, pruritis, and discharge may be present Treat with local excision and adequate margins APR resection is performed for extensive or infiltrating tumors What you might see Ulcerating tumor has a pearly border Skin Cancer Squamous Cell/Epidermoid carcinoma Tumor appears superficial, discrete, and hard Ulcerates with progression Mets to regional lymph nodes can occur Treat with wide local excision What you might see Ulcerating friable tumor is noted Bowen’s disease Intraepidermal squamous cell carcinoma that spreads intraepidermally Precursor to squamous cell carcinoma of the anus Associated with HPV infection Itching and burning, pain and bleeding Treatment wide local excision with frozen section to ensure adequate margins What you might see An indurated erythemato-squamous patch involving the perianal area Extramammary Paget’s Disease Large, round, clear-staining cells with large nuclei Symptoms of ulceration, discharge, pruritis, and occasionally bleeding and pain Treatment depends on the presence/absence or underlying invasive carcinoma Use of retinoid, etretinate, may benefit when there is no invasive carcinoma More infiltrating disease an APR may be needed, otherwise wide local excision with grafting should be adequate for noninvasive disease What you might see Irregular but wellmarginated erythematous erosive patch with slightly indurated edges Extramammary Paget’s Disease Stage I-localized perianal disease without carcinoma-tx with wide local excision Stage IIA-localized disease without underlying malignancy-tx with wide local excision Stage IIB-localized dx with associated anorectal carcinoma-tx with APR Stage III-associated carcinomatous spread to regional lymph nodes-tx with APR plus chemoradiation, possible radical inguinal node dissection Stage IV-distant mets-tx with standard palliative cancer management