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Recurrent Perianal Paget's Disease

Bahar Sevimli Dikicier1*, Berna Solak1, Mahizer Yald?z1, Zeynep Kahyaoglu2 and Teoman Erdem1
  1. Department of Dermatology, Sakarya University Medical School, Turkey
  2. Department of Pathology, Sakarya University Medical School, Turkey
Corresponding Author: Dr. Bahar Sevimli Dikiciero,Sakarya Universitesi E?itim ve Ara?t?rma Hastanesi, Dermatoloji Klini?i, Sakarya, Turkey, Tel: +905326469196 E-mail: [email protected]
Received: November 02, 2015 Accepted: December 09, 2015 Published: December 15, 2015
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Abstract

Changes or disruptions in the process of mandibular development can happen in the early stages of formation, and the malformations may be isolated or part of a syndrome. Hypoplasia or aplasia of the mandibular condyles indicates underdevelopment or non-development and is associated with multiple craniofacial abnormalities. Patients who experience these congenital deformities may benefit from reconstruction using total joint prostheses. In addition, orthognathic surgery may be required to treat severe respiratory syndromes and to correct dentofacial deformities to obtain functional and aesthetic results. Therefore, this paper presents a rare case of aplasia of the mandibular condyles and treatment of dentofacial deformities associated with severe obstructive sleep apnea and the reconstruction of the temporomandibular joint.

Keywords

Extra-mammary perianal Paget's disease (EPPD); Multiple tubulovillous adenomas; Colonoscopy

Introduction

Extra-mammary perianal Paget's disease (EPPD) is a rare skin disorder of unknown aetiology, which is frequently associated with malignancy. Here we report a case of recurrent perianal Paget’s disease which has no association with a malignancy and comments upon its diagnosis and treatment.

Case

A 58 year-old man was referred to our dermatology department because of a perianal skin lesion that was recognized during a colonoscopy procedure which was performed for constipation. His lesion was a 4 cm round plaque which was erythematous at the center with a surrounding rim of whitish membranous appearance in the right perianal area (Figure 1). He had pruritus on that area for years. The patient told that he had a similar condition which was operated two years ago.
Histopathologic examination of his skin biopsy was reported as epidermal infiltration of polygonal atypical cells with round pleomorphic nucleus and prominent nucleoli and wide pale eosinophylic cytoplasm along the basal membrane (Figure 2).
These atypical cells were heavily stained by carsinoembryogenic antigene (CEA) immunohistochemically (Figure 3). No other colonic, intestinal, or urogenital mass or sign of neoplasm was detected by imaging modalities. It was revealed that he had undergone the similar processes 2 years ago. The patient is diagnosed as primary recurrent perianal Paget’s disease (PPD). An operation for a wide local excision was planned.

Discussion

Extramammary Paget's disease can be found in various locations, including the patients typically complain of anal pruritus, bleeding, and discharge [1]. Approximately 12% of patients with anal margin Paget's disease harbor a colorectal neoplasm mandating full colonoscopy and 24% of patients have associating cutaneous adnexal adenocarsinoma [2].
No malignancy was detected in our patient by abdominal ultrasound and computed tomography, a simple polyp was excised during colonoscopy in the rectum, which was reported as fibroid polyp histopathologically.
Pruritus ani was the main complaint of this patient which was present for many years. The patient had the same story 3 years ago which was ended by excision of the lesion on the right perianal area. Multiple tubulovillous adenomas had been resected during work up at that time. Most authors recommend surgery as the treatment of choice [2-4]. Besa et al. recommends radiotherapy for patients not suitable for surgery, or post operatively [5,6]. Amin reported a successfully treated patient with radiotherapy who had a history as reccurring more extensively four times after each excision [7]. A wide excision is planned for our patient.
The differential diagnosis of pruritus ani includes cutaneous candidiasis, tinea cruris, contact dermatitis, intertrigo, lichen simplex chronicus, psoriasis, Bowen disease and even basal cell carcinoma; most of which are seen commonly. PPD is a rare disorder, patients should be examined carefully not to miss the diagnosis. Wide local excision remains the treatment of choice with an evolving role for various adjuvant therapies. Despite adequate resection long term outlook remains guarded due to high recurrence rate.

Figures at a glance

Figure 1 Figure 2 Figure 3
Figure 1 Figure 2 Figure 3

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