Case Report

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Toluidine blue for extramammary Paget’s disease in Mohs micrographic surgery

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Toluidine blue for extramammary Paget’s disease in Mohs micrographic surgery

We report an elderly gentleman with Extramammary Paget’s disease (EMPD) treated with Mohs micrographic surgery (MMS) using Toluidine blue staining intraoperatively as to detect the Paget’s cells. An elderly man presented with an erythematous plaque on the left inguinal fold which showed in-situ EMPD on histopathological examination. Investigations for secondary EMPD were negative and the patient was treated with MMS. During MMS, the specimens from the patient were stained using Toluidine blue in order to detect the Paget cells and to determine the appropriate negative margin. At 4 years follow up the patient is free of recurrence. Toluidine blue is a fast, user-friendly dye that can be used intraoperatively during MMS as to detect Paget cells and thus to determine the appropriate negative margin.

Well-demarcated erythematous plaque on the arm

A 67-year-old Caucasian man presented with an asymptomatic, well-defined, irregular shaped, erythematous scaly plaque on the left upper arm for five years [Figure 1]. There was no cervical or axillary lymphadenopathy. A skin punch biopsy was performed from the plaque.

DISCUSSION

Extramammary Paget disease is a rare intraepidermal adenocarcinoma that primarily affects apocrine gland-bearing skin most commonly over the anogenital area. When the disease affects nonapocrine bearing skin, it is called ectopic extramammary Paget disease.1 The pathophysiology of ectopic extramammary Paget disease is unknown. Sawada et al. reported the possibility that ectopic extramammary Paget disease originates potentially not only from the apocrine gland but also from the eccrine gland.2 Clinically and histologically, there is no difference between ectopic extramammary Paget disease and conventional one.2 Extramammary Paget disease is well known for its association with malignancy. Chanda reported the incidence of underlying adnexal carcinoma and concurrent internal malignancy associated with extramammary Paget disease as 24% and 12%, respectively.3 The location of the underlying internal malignancy appears to correlate with the site of extramammary Paget disease. For example, the perianal and perineal location is associated with adenocarcinoma of the digestive system and genitourinary malignancy, respectively.4

Till now, there have been 46 cases of ectopic extramammary Paget disease in literature, of which only two cases have been reported in association with internal malignancy. The first case is ectopic extramammary Paget disease of the lateral thigh arising in association with sweat gland carcinoma of the same area,5 the second being ectopic extramammary Paget disease in the midline of the abdomen with underlying colon adenocarcinoma.6

Whole-body evaluation for malignancy should be performed. For evaluation of internal neoplasm, positron emission tomography-computed tomography, upper and lower endoscopy and laboratory tests were recommended. However, the patient refused further testing.7

Extramammary Paget disease is clinically similar to Bowen disease and superficial basal cell carcinoma.8 Thus, clinicians should keep in mind the possibility of ectopic extramammary Paget disease when there is treatment resistance skin lesion with eczematous morphology on the apocrine gland poor regions.

The diagnosis of extramammary Paget disease, in this case, was supported by immunohistochemical findings. Pagetoid cells in pagetoid Bowen disease usually demonstrate immunohistochemical features of squamous cells, which are negative for CK7 and positive for P63.9 In this case, positivity for CK7 but negativity for p63 and Melan A supported the diagnosis of extramammary Paget disease, ruling out Bowen disease and melanoma in situ. A negative result of CK20 immunohistochemical stain supported the diagnosis of primary extramammary Paget disease rather than secondary extramammary Paget disease from an internal malignancy.9 This case has been reported as a rare case of ectopic extramammary Paget disease appearing on an unusual site.

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Co-occurrence of apocrine adenocarcinoma and invasive mammary-type ductal carcinoma in extramammary Paget disease of the axilla

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Co-occurrence of apocrine adenocarcinoma and invasive mammary-type ductal carcinoma in extramammary Paget disease of the axilla

Extramammary Paget disease (EMPD) is an uncommon malignancy that occurs in apocrine gland-rich areas of the body. EMPD of the axilla is rare, but a few cases have been reported. Some cases of EMPD have been reported with underlying apocrine adenocarcinoma; rarely, mammary-type ductal carcinoma can accompany EMPD. Here, we report a very rare case of EMPD with apocrine adenocarcinoma and invasive mammary-type ductal carcinoma.

Vulvar Paget’s disease presenting on the background of clinically diagnosed lichen sclerosus

Vulvar Paget’s disease presenting on the background of clinically diagnosed lichen sclerosus

61-year-old woman was referred to the outpatient’s department due to persistent vulvar itchiness and soreness. She had been clinically diagnosed with lichen sclerosus (LS) in primary healthcare two years before referral and was being treated with topical steroids. year before her presentation, she was diagnosed with breast cancer and had a lumpectomy, axillary lymph node dissection, and adjuvant radiotherapy. She had a history of hysterectomy for benign reasons, suffered from pernicious anemia and was a non-smoker. panel board in our case, due to overlapping symptoms with LS there can be a time delay before the diagnosis of VPD is established through vulvar biopsies.

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R Inguinal/R Scrotum Extramammary Paget’s Disease with Diffuse Spine Metastasis Complicated by Microangiopathic Hemolytic Anemia

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R Inguinal/R Scrotum Extramammary Paget’s Disease with Diffuse Spine Metastasis Complicated by Microangiopathic Hemolytic Anemia

A 47-year-old male presented with a groin lesion in 2011. Initial treatment with antifungals and vinegar was unsuccessful. In 2016, biopsy of this lesion was pursued with diagnosis of extramammary Paget’s disease (EMPD). Prior to the scheduled excision, he developed constant lower back pain with radiation to his lower extremities. MRI confirmed vertebral metastasis. Despite surgical and radiation therapy, his back pain progressed, and repeat imaging showed epidural spread of tumor in the thoracic spine. Later, the patient was admitted to the hospital due to severe anemia and thrombocytopenia. Further work-up confirmed the diagnosis of microangiopathic hemolytic anemia (MAHA). As we know, there are only few reports of spinal metastases in patients with EMPD. To the best of our knowledge, this is the first case report of EMPD complicated by MAHA.

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Multicentric Primary Extramammary Paget Disease: A Toker Cell Disorder?

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Multicentric Primary Extramammary Paget Disease: A Toker Cell Disorder?

Toker cells are epithelial clear cells found in the areolar and nipple areas of the breast, vulvar region, and other apocrine gland–bearing areas of the skin. Toker cells have been implicated in the pathogenesis of clear cell papulosis, cutaneous hamartoma with pagetoid cells, and rare cases of primary extramammary Paget disease (EMPD) but not in secondary EMPD with underlying adenocarcinoma. The pathogenesis of primary EMPD is not well defined. We report a case of multicentric primary EMPD with evidence of Toker cell proliferation and nonaggressive biologic behavior in a 63-year-old white man. A detailed description of the morphologic and biologic features of Toker cells and their possible carcinogenetic links also are discussed. Based on the observation and follow-up of our patient, we hypothesize that multicentric primary EMPD starts with Toker cell hyperplasia and can potentially evolve to carcinoma in the genital region.

Extramammary Paget disease (EMPD), which was first described by Crocker1 in a patient with erythematous patches on the penis and scrotum, is morphologically identical to mammary Paget disease (MPD) of the nipple. The principal difference between EMPD and MPD is anatomic location.

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Apocrine Carcinoma of the Groin Possibly Associated with Extramammary Paget's Disease

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Apocrine Carcinoma of the Groin Possibly Associated with Extramammary Paget's Disease

Apocrine carcinoma is a rare malignancy with invasive potential. It presents as painless, slow-growing, firm or cystic, red nodules with focal ulcerations. The tumor is capable of hematogenous dissemination to the liver, lungs, and bone as well as lymphatic spread. In addition, apocrine carcinomas cause intra-epidemial pagetoid spread. We report a case of an apocrine carcinoma related with extensive extramammary Paget's disease (EMPD). The relationship between apocrine carcinoma and EMPD remains to be understood. Co-existing cases with apocrine carcinoma and EMPD are discussed to better understand the relationship between these two malignant apocrine tumors.

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Failure of Extensive Extramammary Paget Disease of the Inguinal Area to Clear With Imiquimod Cream, 5%

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Failure of Extensive Extramammary Paget Disease of the Inguinal Area to Clear With Imiquimod Cream, 5%

Of the 27 published cases that describe imiquimod treatment of EMPD, 6 report treatment failure (22%), but factors that may contribute to treatment failure are not well understood. In the present patient, treatment with imiquimod may have been complicated by variable lesion thickness, which inhibited uniform penetration of imiquimod, or the presence of invasive disease not detected on initial biopsy. The efficacy of imiquimod to treat extensive invasive EMPD has not been demonstrated, and surgical approaches remain the most appropriate treatment for invasive disease. Variable responses to topical imiquimod use among patients suggest that other factors may be important in determining response to therapy.

Surgical management of extramammary Paget disease (EMPD) remains a therapeutic mainstay, but alternative treatments for primary limited cutaneous EMPD in the anogenital area that avoid cosmetic and functional defects after extensive tissue removal are under investigation. Local recurrence of EMPD can be significant,1 highlighting the insidious nature of EMPD and the need to identify more effective treatments. The topical immunomodulator imiquimod, 5%, has been reported to induce clinical and histologic resolution of superficial EMPD,2-12 but several cases of imiquimod failure have also been described.13-17 Herein, we present a case of genital EMPD considered to be limited to in situ disease at the beginning of treatment with topical imiquimod that proved refractory, with demonstration of invasive disease during treatment. We also review the current literature (articles published in English and obtained through PubMed, Ovid, and GoogleScholar searches conducted between July 1, 2009, and August 31, 2010) regarding successes (n = 21) and failures (n = 6) of imiquimod therapy for EMPD and present characteristics that could portend treatment failure.

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Extramammary Paget’s Disease: 20 Years of Experience in Chinese Population

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Extramammary Paget’s Disease: 20 Years of Experience in Chinese Population

Extramammary Paget’s disease is an uncommon intra-epidermal carcinoma of apocrine gland-bearing skin. The most frequently involved anatomical sites include the scrotum, penis, vulva, and perineal and perianal region. Other rare sites of involvement, such as the eyelids [1], axilla, and external auditory canal [2], have been reported. There have been case reports describing ectopic EMPD occurring in areas devoid of apocrine gland, but they are exceedingly rare [3].

In the majority of the cases, the disease is limited to the epidermis. However, it is well known that EMPD has the potential of dermal invasion [4]. Moreover, its association with underlying internal malignancies remained one of the most interesting characteristics of the disease. Data in the literature come from small series and case reports only, especially on Chinese patients.

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Successful Intra-Arterial Chemotherapy for Extramammary Paget’s Disease of the Axilla in a Patient with Parkinson’s Disease

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Successful Intra-Arterial Chemotherapy for Extramammary Paget’s Disease of the Axilla in a Patient with Parkinson’s Disease

Extramammary Paget’s disease (EMPD) is a rare intraepithelial neoplasm occurring less frequently in men and even more rarely in the axilla. A 59-year-old man with severe Parkinson’s disease presented with axillary EMPD. The neurological comorbidity made treatment of the EMPD problematical and prompted us to propose locoregional intra-arterial chemotherapy in single short sessions. Two innovative chemotherapeutic macrocomplexes were used: doxorubicin incorporated in large liposomes and the taxane paclitaxel incorporated in albumin nanoparticles. A therapeutic response was seen right from the first treatment and was macroscopically close to complete after four cycles. Five months after the end of treatment the patient had minimal visible disease and had enjoyed a distinct improvement in quality of life, with no noteworthy complications related to the intra-arterial chemotherapy with percutaneous transfemoral catheterization.

Full Recovery of Recurrent Extramammary Paget’s Disease of the Vulva (REMPD-V) After Imiquimod Treatment (PDF)

Full Recovery of Recurrent Extramammary Paget’s Disease of the Vulva (REMPD-V) After Imiquimod Treatment (PDF)

Although rare, extramammary Paget’s disease (EMPD) is a serious condition because underlying internal malignancy may accompany super cial cutaneous lesions.Extramammary Paget disease is characterised by a chronic eczema-like rash of the skin around the anogenital regions of males and females. Under the microscope it looks very similar to the more common type of mammary Paget ́s disease that occurs on the breast. Extramammary Paget disease most commonly occurs in women aged between 50-60 years. Although surgical excision is the generally accepted standard of care for EMPD. The EMPD-V recurrence rates are high despite aggressive surgical intervention, treatment with topical imiquimod 5 percent cream has reportedly been ef cacious in clearing lesions. We report the case of a 72-year-old woman with biopsy-proven EMPD-V of the thigh treated successfully with imiquimod application thrice weekly for 6 weeks.

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Treatment of limited extent extramammary Paget's Disease with 5 percent imiquimod cream

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Treatment of limited extent extramammary Paget's Disease with 5 percent imiquimod cream

Although there is not extensive experience with the use of topical imiquimod 5 percent in the treatment of EMPD, the reported cases of successful use of this agent in limited extent EMPD are promising. Larger scope, randomized controlled trials are needed to determine the true safety and efficacy of imiquimod in comparison to the other therapy modalities currently used to manage EMPD. To the best of our knowledge, the treatment of EMPD of the thigh with imiquimod is not reported elsewhere in the literature, and we report our case as further evidence of the potential role imiquimod may play in this disorder.

Extramammary Paget's Disease Resistant to Surgery and Imiquimod Monotherapy But Responsive to Imiquimod Combination Topical Chemotherapy With 5-Fluorouracil and Retinoic Acid: A Case Report

Extramammary Paget's Disease Resistant to Surgery and Imiquimod Monotherapy But Responsive to Imiquimod Combination Topical Chemotherapy With 5-Fluorouracil and Retinoic Acid: A Case Report

Extramammary Paget's disease (EMPD) is an uncommon skin neoplasm that usually affects the elderly population and occurs in the genital, anorectal, or axillary areas. The recommended treatment of EMPD involves surgical excision, including Mohs micrographic surgery; however, surgery is associated with a high rate of recurrence. There have been reports of successful treatment of recurrence with monochemotherapy involving topical imiquimod 5% cream. We report a case of EMPD recurrence after surgery that was resistant to imiquimod monotherapy but that completely resolved after imiquimod was combined with topical 5-fluorouracil (5-FU) and retinoic acid. To our knowledge, this is the first reported case of imiquimod combination therapy with 5-FU and retinoic acid for the treatment of recurrent EMPD.

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