Mammary Paget's Disease Presenting as an Annular Plaque.

IF 0.6 4区 医学 Q4 DERMATOLOGY
Acta Dermatovenerologica Croatica Pub Date : 2022-12-01
Chinatsu Matsumoto, Shiro Niiyama, Takuya Nagata, Toshiaki Oharaseki, Hidetsugu Fukuda
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Abstract

Dear Editor,Mammary Paget's disease (MPD) is an adenocarcinoma localized within the epidermis of the nipple and/or the areola of the breast, and it is as a rule associated with a carcinoma of the underlying lactiferous ducts, where it usually starts. MPD is relatively rare, observed in 0.7-4.3% of all breast cancers (1). We present a patient with MPD and atypical clinical finding as an annular plaque. A 74-year-old Japanese woman with a past medical history of hypothyroidism presented with a 6-month history of an itching plaque on the left areola. The patient had been treated with the application of topical steroids for a duration of approximately 5 months, and showed no clinical improvement. Physical examination showed a pink plaque encircling the nipple on the left areola (Figure 1, a). The right nipple and areola appeared normal (Figure 1, b). No palpable masses were detected within either breast. A 3.5 mm punch biopsy of the skin at the 6 o'clock position of the left areola was performed. Histological examination showed single and small aggregations of atypical cells with large hyperchromatic nuclei and pale-staining, ample cytoplasm throughout the epidermis. There was a lymphocytic infiltration in the dermis (Figure 1, c). Immunohistochemical studies were positive for CK7 and negative for S-100 and HMB45. With the diagnosis of MPD, the patient underwent a partial mastectomy of the left breast center area, consisting of surgical excision of the left nipple, the adjacent surrounding areolar skin, and subcutaneous tissues. Subsequently, radiation therapy for the residual breast was prepared. As has been described in detail by Kanitakis, the skin lesion develops insidiously as a scaly, fissured, or oozing erythema of the nipple and, more rarely, the areola. Advanced lesions present as a well-demarcated, round, ovoid, or polycyclic eczema-like plaque with a pink or red hue. It is occasionally slightly infiltrated and has an erosive, oozing, scaly, or crusted surface. The lesions are almost invariably unilateral, showing centrifugal spread. Retraction or ulceration of the nipple are often noted (1). The present case exhibited a very rare clinical finding of a plaque encircling the nipple, which has not been reported previously. It was initially difficult to establish the diagnosis of MPD, and biopsy was needed to obtain a definitive diagnosis. Differential diagnosis of MPD comprises eczema as atopic dermatitis or contact dermatitis, erosive adenomatosis, and malignant skin condition such as Bowen's disease, superficial basal cell carcinoma, or superficially spreading melanoma. As in the present case, individuals presenting with an annular plaque are often considered to have sebaceous hyperplasia. Sebaceous hyperplasia is a common, benign skin condition involving hypertrophy of the sebaceous glands, common in middle-aged or older adults (2). These lesions can be single or multiple and manifest as yellow, soft, small papules. These papules are occasionally seen around the nipple, forming an annular plaque. In general, sebaceous hyperplasia is described as yellow-colored papules among Caucasians. However, caution is needed, since it is characterized by skin-colored papules among some Asians.In the present case, some pigmentation (2 to 3 mm in diameter) was observed on the left nipple. Pigmented MPD have been reported, and the mechanism underlying the pigmentation is not yet fully understood, but it has been proposed that Paget cells may release melanocytic chemoattractants or basic fibroblast growth factors that stimulate the proliferation of melanocytes within the tumor nests (3). The possibility of physiological pigmentation cannot be ruled out in the present case; on the other hand, the possibility of pigmented MPD cannot be ruled out either, since no pigmentation was observed on the right nipple.

乳腺佩吉特病表现为环状斑块。
亲爱的编辑,乳腺佩吉特病(MPD)是一种局限于乳头表皮和/或乳房乳晕的腺癌,通常与底层乳管癌有关,它通常是从这里开始的。MPD相对罕见,在所有乳腺癌中占0.7-4.3%(1)。我们报告了一例MPD患者,其非典型临床表现为环形斑块。74岁日本女性,既往有甲状腺功能减退病史,左侧乳晕瘙痒斑块6个月。患者局部应用类固醇治疗约5个月,临床无改善。体格检查显示左侧乳晕有粉红色斑块环绕乳头(图1,a)。右侧乳头和乳晕正常(图1,b)。双侧乳腺均未发现可触及的肿块。在左侧乳晕6点钟位置对皮肤进行3.5 mm穿孔活检。组织学检查显示单个和小的非典型细胞聚集,细胞核大而深染,染色苍白,整个表皮有丰富的细胞质。真皮中有淋巴细胞浸润(图1,c)。免疫组化研究显示CK7阳性,S-100和HMB45阴性。诊断为MPD后,患者行左侧乳房中心部分切除术,包括左乳头、邻近周围乳晕皮肤和皮下组织的手术切除。随后,准备对残乳进行放射治疗。正如Kanitakis详细描述的那样,皮肤病变发展为乳头的鳞状,裂隙或渗出性红斑,更罕见的是乳晕。晚期病变表现为界限分明、圆形、卵形或多环湿疹样斑块,呈粉红色或红色。偶尔有轻微浸润,表面有侵蚀、渗出、鳞片或结壳。病变几乎都是单侧的,呈离心扩散。乳头缩回或溃疡常被注意到(1)。本病例表现出一个非常罕见的临床发现,斑块环绕乳头,这在以前没有报道过。最初很难确定MPD的诊断,需要活检来获得明确的诊断。MPD的鉴别诊断包括湿疹,如特应性皮炎或接触性皮炎,糜烂性腺瘤病和恶性皮肤病,如Bowen病,浅表基底细胞癌或浅表扩散黑色素瘤。在本病例中,出现环状斑块的个体通常被认为患有皮脂腺增生。皮脂腺增生是一种常见的良性皮肤疾病,包括皮脂腺肥大,常见于中老年人(2)。这些病变可为单个或多个,表现为黄色、柔软的小丘疹。乳头周围偶尔可见丘疹,形成环状斑块。一般来说,白种人的皮脂腺增生表现为黄色丘疹。然而,需要谨慎,因为一些亚洲人的特点是皮肤颜色的丘疹。在本病例中,在左乳头上观察到一些色素沉着(直径2至3mm)。色素沉着性MPD已被报道,其色素沉着的机制尚不完全清楚,但有人提出Paget细胞可能释放黑色素细胞化学引诱剂或碱性成纤维细胞生长因子,刺激肿瘤巢内黑色素细胞的增殖(3)。在本病例中,不能排除生理性色素沉着的可能性;另一方面,由于右乳头未见色素沉着,也不能排除色素沉着的可能性。
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来源期刊
Acta Dermatovenerologica Croatica
Acta Dermatovenerologica Croatica 医学-皮肤病学
CiteScore
0.60
自引率
0.00%
发文量
23
审稿时长
>12 weeks
期刊介绍: Acta Dermatovenerologica Croatica (ADC) aims to provide dermatovenerologists with up-to-date information on all aspects of the diagnosis and management of skin and venereal diseases. Accepted articles regularly include original scientific articles, short scientific communications, clinical articles, case reports, reviews, reports, news and correspondence. ADC is guided by a distinguished, international editorial board and encourages approach to continuing medical education for dermatovenerologists.
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