Indian Journal of Dermatology
IJDŽ QUIZ
Year
: 2013  |  Volume : 58  |  Issue : 5  |  Page : 402--403

Greyish verrucous plaque with erosions on the perianal area


Mahendra M Kura, Vrushali K Rane, Virendra D Singh 
 Department of Dermatology, Venereology and Leprosy, Grant Medical College, Mumbai, India

Correspondence Address:
Mahendra M Kura
Department of Dermatology, Venereology and Leprosy, Grant Medical College, Mumbai
India




How to cite this article:
Kura MM, Rane VK, Singh VD. Greyish verrucous plaque with erosions on the perianal area.Indian J Dermatol 2013;58:402-403


How to cite this URL:
Kura MM, Rane VK, Singh VD. Greyish verrucous plaque with erosions on the perianal area. Indian J Dermatol [serial online] 2013 [cited 2017 Apr 25 ];58:402-403
Available from: http://www.e-ijd.org/text.asp?2013/58/5/402/117330


Full Text

A 70-year-old male presented with a mildly pruritic elevated lesion discharging sticky serous fluid in perianal region since 2 years. The lesion had progressed despite local applications of 25% podophyllin, steroids, and antifungal agents. There was no history of bowel or bladder disturbances, weight loss, or homosexual practices.

Dermatological examination revealed a grayish, sharply marginated verrucous plaque with superficial erosions present circumferentially around the anal opening. Per rectum examination revealed its extension into the anal canal at 3, 6, and 12 O'clock position [Figure 1] but not beyond the margins of the anal canal. There was no regional or distant lymphadenopathy. Skin biopsy revealed hyperkeratosis, papillomatosis, and irregular acanthosis. Numerous large cells with pale cytoplasm and hyperchromatic nucleus were seen in epidermis [Figure 2], few of which were also extending into the hair follicle [Figure 3]. Surgical opinion and ultrasonography of abdomen and pelvis ruled out bowel involvement.{Figure 1}{Figure 2}{Figure 3}

 Question



 What is your diagnosis?



 View AnswerAnswer

Primary Extramammary Pagets disease

Diagnosis

Depending on the history, classical lesion at susceptible site, presence of classical Paget's cells in the epidermis, and in the absence of any systemic involvement, patient was finally diagnosed as a case of Primary extramammary Paget's disease (EMPD).

Review

EMPD is an uncommon neoplastic condition of apocrine-bearing skin. Lesions of EMPD typically involve the vulvar (the most common site affected), perianal, perineal, scrotal, and penile regions. Two major types of EMPD are described. In the first type called "primary" EMPD, constituting 75% cases, the disease originates in the skin either arising in situ epithelium or adnexal carcinoma. Pathogenesis of primary EMPD still remains elusive, but it is hypothesized that pluripotential germinative cells within the epidermis go awry in their attempt to form apocrine structures and result in the formation of malignant Paget's cells. [1],[2] In the second type "secondary" EMPD, noticed in 25% cases, contiguous epithelial spread form a visceral malignancy of either genitourinary or gastrointestinal. [1],[2],[3]

Most cases of vulvar EMPD are of primary type, but EMPD of the male genitalia is frequently associated with internal malignancy of genitourinary tract. Perianal EMPD, in both sexes, is associated with anal or colorectal carcinoma in 70-80% of cases. [1],[2],[3] However in our case, no underlying malignancy was detected, despite the lesion being on the susceptible site.

Most of the cases reported are in seventh decade although the age of presentation ranges between 50 and 80 years and females are affected more frequently than males. [1] The disease most commonly presents as gradually progressing, well-marginated, erythematous or leucoplakic plaques with eczematous changes, usually associated with intermittent itching, but there might be variable degree of burning, irritation, tenderness, bleeding, oozing, maceration, and swelling .[1] In most of the cases, there is history of progression of the lesions over months/years despite being treated with topical therapy for various clinical differentials as was noticed in our case.

The diagnosis of EMPD is based on characteristic history, typical location and morphology of the lesions, and histopathology and histochemistry findings. Histopathology reveals distinctive intraepidermal neoplastic proliferative cells (Paget's cells) with abundant pale-staining cytoplasm and large atypical nuclei. In primary EMPD, Paget's cells may be present in the outer root sheath (as in our case) and is characteristic, but are also found in sebaceous, apocrine, or eccrine gland structures. Immunohistochemical stains such as carcinoembryonic antigen, cytokeratin 3,7,20, and S-100 are helpful in diagnosing EMPD and its differentiation into primary and secondary variants. [1],[4]

Clinical differential diagnosis of EMPD in anogenital area includes psoriasis, lichen planus, seborrheic dermatitis, lichen simples chronicus, verruca vulgaris, periorificial tuberculosis, tuberculous verrucosa cutis, Bowen's disease, mycosis fungoides, malignant melanoma, and dermatophyte infection. Histopathological differentials include pagetoid Bowen'disease, and pagetoid malignant melanoma in situ.

Although surgical excision carries a high recurrence rate, it remains the treatment of choice for EMPD. Imiquimod is a promising therapeutic option for the treatment of limited cutaneous EMPD. Treatment-associated morbidity is minimal and self-application improves patient convenience. [1],[5]

Prognosis for primary intraepithelial EMPD is good when compared with the secondary EMPD.

The names of the winners(first three correct entries) are:

Vishal Gupta, New Delhi Riti Bhatia, New Delhi Vinay K, Chandigarh

"Others who have sent correct answers are mentioned below:"

Geeti Khullar, Piyush Kumar, Urmi Khanna, Ganesh Avhad, Resham Vasani, Anupama, Keshavmurthy Adya, Himanshu Gupta, Sudhir Nayak U.K., Vishal Chugh, Tirthankar Gayen, Bhavana R. Doshi, Joginder Kumar, Hari Kishan Kumar.Y., Soumyajit Roychaudhuri.

References

1Lloyd J, Flanan AM. Mammary and Extramammary Paget's disease. J Clin Pathol 2000;53:742.
2Lupton GP, Graham JH. Mammary and Extramammary Paget's disease. In: Friedman RJ, Rigel DS, Kopf AW, Harris MN, Baber D, editors. Cancer of the Skin. Philadelphia: WB Saunders; 1991. p. 217.
3Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C. Paget's disease of the vulva: Prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol 1999;180:4.
4Nowak MA, Guerriere-Kovach P, Pathan A, Campbell TE, Deppisch LM. Perianal Paget's disease: Distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. Arch Pathol Lab Med 1998;122:1077-81.
5Zamponga JC, Flowers FP, Roth WI, Hassenein AM. Treatment of primary limited cutaneous extramammary Paget's disease with imiquimod monotherapy: Two case reports. J Am Acad Dermatol 2002;47:229-35.