Tưởng viêm da suốt 2 năm, người đàn ông phát hiện ung thư hiếm gặp vùng kín

Tưởng viêm da suốt 2 năm, người đàn ông phát hiện ung thư hiếm gặp vùng kín

TS.BS Nguyễn Hồng Sơn - Trưởng khoa Phẫu thuật Tạo hình thẩm mỹ và Phục hồi chức năng, Bệnh viện Da liễu Trung ương cho biết, thời gian gần đây bệnh viện tiếp nhận một số trường hợp mắc bệnh Paget sinh dục nhưng được phát hiện muộn do nhầm lẫn với các bệnh da liễu thông thường.

Điển hình là nam bệnh nhân 60 tuổi sống chung với tổn thương vùng sinh dục suốt 2 năm vì cho rằng chỉ bị viêm da thông thường. Chỉ đến khi tổn thương lan rộng, trợt loét, chảy dịch nhiều và không đáp ứng điều trị, người bệnh mới đến cơ sở chuyên khoa thăm khám và được chẩn đoán mắc bệnh Paget sinh dục – một dạng ung thư biểu mô tuyến hiếm gặp.

Qua khai thác bệnh sử, người bệnh cho biết các sẩn đỏ vùng bìu xuất hiện từ lâu nhưng không đau, không ngứa. Trong quá trình điều trị trước đó, bệnh nhân từng được chẩn đoán viêm da bìu và sử dụng nhiều loại thuốc uống, thuốc bôi nhưng tình trạng không cải thiện.

Aurélie Paget doit se battre contre une maladie rare qui porte son nom

Aurélie Paget doit se battre contre une maladie rare qui porte son nom

At 37, Aurélie Paget is living with a rare disease… one that bears her own name: Paget’s disease. After four surgeries and months of uncertainty, she is now facing a recurrence. As she prepares to begin radiotherapy and chemotherapy, she hopes to raise awareness of the illness that is consuming her.

Poland nurse practitioner confronts one of the world's rarest cancers

Poland nurse practitioner confronts one of the world's rarest cancers

For 20 years, nurse practitioner Becky Topham has been the one tending to patients in need.

Now, the 44-year-old mother of two from Poland finds herself on the other side of the hospital bed, facing a diagnosis so rare that only 300 people are affected each year.

Topham is battling Extramammary Paget’s Disease (EMPD), an aggressive form of skin cancer that often goes misdiagnosed in its early stages. What she thought was a mild but nagging skin issue eventually led to a biopsy, and in May, doctors confirmed the diagnosis.

Extramammary Paget disease (EMPD): What you need to know

Extramammary Paget disease (EMPD): What you need to know

Extramammary Paget disease (EMPD) is a very rare form of skin cancer, affecting an estimated one in a million people in the U.S. It tends to grow slowly, but if left untreated, it can become invasive and spread to other parts of the body. That’s why early detection is so important.

Because it’s so rare, many doctors may never see a case in their careers. To help us understand more, we spoke with Phyu Aung, M.D., Ph.D., a dermatopathologist who specializes in rare skin cancers like EMPD.

Extramammary Paget’s Disease, The Rare Skin Condition That’s Often Misdiagnosed—Are You at Risk?

Extramammary Paget’s Disease, The Rare Skin Condition That’s Often Misdiagnosed—Are You at Risk?

The majority of skin irritations are ignored and dismissed as being caused by simple dryness or reactions to clothing or heat. However, the cause may be noticeably more serious for people who have lesions or persistent itching in intimate areas, especially those over 60. Despite being uncommon, extramammary Paget’s disease is becoming more widely acknowledged for its role in dermatological misdiagnosis. It’s a condition that often lurks under the benign exterior of common dermatitis, rarely making a big splash.

Virginia’s Rare Cancer Journey: Extramammary Paget’s Disease

Virginia’s Rare Cancer Journey: Extramammary Paget’s Disease

Even though I was not a stranger to chronic illness, cancer was a whole different ballgame.

It all started with some itching. I thought I had a rash that was transient, and I couldn’t make an appointment quick enough to catch it because it would be gone in a day or two. My 2023 well-woman visit finally lined up to a day I had the issue occurring. I mentioned to my gynecologist that I had felt some itching off and on for a few months, and that I was itching that day.

Extramammary Paget's Disease Research

Extramammary Paget's Disease Research

Extramammary Paget's Disease (EMPD) is a rare malignancy of the skin, primarily affecting the genital and perianal regions in older adults, often linked to underlying internal malignancies. Recent advancements in research emphasize improved diagnostic methods, including multiple biopsies and immunohistochemical markers like TRPS1, which aid in distinguishing EMPD from similar conditions. Evidence-based clinical guidelines recommend surgical resection, particularly Mohs micrographic surgery (MMS), as the preferred treatment due to its lower recurrence rates compared to wide local excision (WLE). Non-invasive EMPD may benefit from therapies like imiquimod, a topical immune response modifier, which has shown promise in achieving remission despite side effects such as fatigue and headaches. These developments underscore the importance of personalized treatment strategies and ongoing evaluation of patient outcomes to improve the management of EMPD.

Anatomic Location-Specific Screening for Adenocarcinoma in EMPD

Anatomic Location-Specific Screening for Adenocarcinoma in EMPD

The Extramammary Paget’s disease (EMPD) was associated with underlying internal adenocarcinoma, known as secondary EMPD, though differences by an anatomic subtype and screening implications remained unclear.  

Researchers conducted a retrospective study to determine the rates of secondary EMPD and the types of adenocarcinomas associated with EMPD anatomic subtype and to propose a screening algorithm for underlying the adenocarcinoma.  

Investigators concluded that screening for underlying the adenocarcinoma in EMPD should be guided by an anatomic location.

下身現紅疹痕癢以為係濕疹？6個地方現紅疹小心患罕見癌症

下身現紅疹痕癢以為係濕疹？6個地方現紅疹小心患罕見癌症

70歲的黃伯伯近日發現下身位置出現一塊紅疹，本來不痕不痛，以為只是自己較少清洗這位置的皮膚所致，於是不太理會。但到一年後見紅疹慢慢變大，間中也有些痕癢，擔心可能演變成濕疹，才去求醫。醫生發現有懷疑，外觀上與一般皮膚發炎有異，於是為黃伯伯進行切皮化驗，才確診那是較罕見的乳房外佩吉特氏病（Extramammary Paget's disease），是一種罕見的癌症

Barry Humphries: Comic battled inoperable EMPD cancer for years

Barry Humphries: Comic battled inoperable EMPD cancer for years

Barry Humphries fought a secret battle with inoperable cancer for years before his death last week.

The Australian actor and comic, known for his alter ego Dame Edna Everage, died aged 89 on April 22 following complications from a recent hip replacement surgery.

“Close family sources” confirmed to the The Saturday Telegraph he had been diagnosed with Extramammary Paget’s disease, a rare form of skin cancer, in 2021 after noticing “something unusual” on one of his testicles when he was in the shower.

Review of Gender Disparities in Initial Treatment Approaches for Genital Extramammary Paget Disease in the US

Review of Gender Disparities in Initial Treatment Approaches for Genital Extramammary Paget Disease in the US

Genital extramammary Paget disease (gEMPD) is a rare skin cancer that affects the genital area. Surgery is the standard treatment for gEMPD. For a study, researchers sought to investigate whether the treatment and outcomes of gEMPD differ by sex and US region. The researchers found that women were more frequently offered total skinning procedures for gEMPD, while men were more frequently offered MMS.

Malattia extramammaria di Paget: linee guida cliniche

Malattia extramammaria di Paget: linee guida cliniche

Sviluppare un set completo di raccomandazioni per la diagnosi e la terapia della malattia extramammaria di Paget nelle persone adulte: è questo l’obiettivo delle linee guida messe a punto da oltre cinquanta ricercatori sotto il coordinamento di Nour Kibbi, del Dipartimento di Dermatologia della Stanford University School of Medicine, Stati Uniti. Ai lavori hanno preso parte, fra gli altri, la Yale School of Medicine, il Memorial Sloan Kettering Cancer Center di New York, la Feinberg School of Medicine di Chicago, la Harvard Medical School di Boston e la Mayo Clinic di Rochester, oltre a clinici britannici, canadesi, australiani, francesi, greci, giapponesi e coreani. La malattia extramammaria di Paget (EMPD) è una rara forma di adenocarcinoma intraepiteliale, con potenziale metastatico, che colpisce tipicamente l’area genitale e perineale femminile e maschile, ma anche inguine, ascelle, gambe, braccia, palpebre, cuoio capelluto, naso, tempie e canale uditivo esterno.

EMPD Interdisciplinary Management Using MMS-CK7 Immunostains

EMPD Interdisciplinary Management Using MMS-CK7 Immunostains

For a study, researchers sought to assess the local recurrence rates and patient-reported outcomes when extramammary Paget’s disease (EMPD) is treated with Mohs micrographic surgery with cytokeratin-7 immunostains (MMS-CK7).

In-depth internal malignancy screening, low local recurrence rates, and high patient satisfaction were all characteristics of interdisciplinary teams that successfully treat EMPD.

After noticing “something weird” in the shower, Barry Humphries (AKA Dame Edna Everage), was diagnosed with extramammary Paget’s disease (EMPD).

After noticing “something weird” in the shower, Barry Humphries (AKA Dame Edna Everage), was diagnosed with extramammary Paget’s disease (EMPD).

After noticing “something weird” in the shower, Barry Humphries was diagnosed with a rare malignancy.

After detecting something weird with his testicles in the shower, BARRY HUMPHRIES realized he had a rare form of skin cancer.

Barry Humphries, better known as Dame Edna Everage, has revealed that he was diagnosed with Paget’s Disease, a type of skin cancer. Following the finding, the Australian actor revealed that he had surgery.

Extra-mammary Paget’s disease survivor: Time matters

Extra-mammary Paget’s disease survivor: Time matters

In 2013, Sandy Fontan was a 53-year-old marathon runner in tip-top shape. She maintained a healthy diet and ran between 25 and 30 miles a week. Then, one day, she noticed a little bump on her anus. It didn’t hurt or bleed, so she thought it was a hemorrhoid. She watched it for more than a year before mentioning it to her gynecologist.

Today, Sandy wishes she’d mentioned it sooner. Because that little bump turned out to be something more serious: an extremely rare type of cancer called extra-mammary Paget’s disease, which usually appears on the skin of the breast.

Key signaling pathway in the pathogenesis of Paget's disease identified

Key signaling pathway in the pathogenesis of Paget's disease identified

Researchers from the University of California, Irvine, in collaboration with their colleagues from Beijing, China, have gained new insights into the development of poorly understood malignant skin cancers collectively known as Paget's disease. The team identified new biomarker genes that highly correlate with Paget's disease. The group then used a Food and Drug Administration (FDA) approved drug to target one of the newly identified molecular features of Paget's disease, leading to prominently improved clinical symptoms in a small cohort of Chinese patients. Their results have been published online in Cell Research.

On the Cusp of Extraordinary Advances in Extramammary Paget Disease

On the Cusp of Extraordinary Advances in Extramammary Paget Disease

I always include extramammary Paget disease (EMPD) and Bowen disease in my differential diagnosis of anogenital rashes for fear of missing them. It is good form to biopsy such lesions should they not respond to standard therapies for the more likely diagnoses in this region (tinea, Candida, seborrheic dermatitis, or psoriasis). Even with this prudent approach, I have diagnosed EMPD only a few times in four decades of practice.

A very strange and endlessly complicated organism

A very strange and endlessly complicated organism

Up until the late spring of 2019, I figured that for a relatively old codger, I was in pretty good health.

I took no medications, my blood pressure and weight were fine, I very seldom had a cold and hadn’t had the flu in many years.

But as an example of how one’s health can take an unexpected turn, I developed a groin rash (the crude term is “jock itch”) that defied the prescription cream that my doctor, Joseph Zeno, seemed to believe would cure the problem.

After some months of using this cream during which I also experienced a general feeling of malaise, Dr. Zeno said, “We have to get to the bottom of this… I’m going to send you to a dermatologist.”

Hoping to find other patients, he revealed a cancer often mistaken for ‘jock itch’

Hoping to find other patients, he revealed a cancer often mistaken for ‘jock itch’

Stephen Schroeder figured he had little to lose, his growing sense of desperation fueled by the loneliness of his unusual diagnosis.

For more than two years, Schroeder had been coping with an extremely rare, invasive cancer called extramammary Paget’s disease (EMPD), which had invaded his scrotum, requiring multiple surgeries. Women account for roughly half of EMPD cases; the cancer, often misdiagnosed as eczema or contact dermatitis, attacks the sweat-producing apocrine glands, including those in the genital and anal areas.

The slow-growing cancer, which in men is frequently misdiagnosed as “jock itch” — slang for a fungal infection — can be fatal. And while treatment is often grueling, for Schroeder the worst part was his sense of isolation: He had never spoken to anyone who shared his diagnosis.

But in time Schroeder would engineer a solution to his isolation, reaping its benefits by connecting with others in similar circumstances. The richness of that experience, he said, has exceeded anything he could have imagined.

© myEMPD.com • Helping those with extramammary Paget’s disease (EMPD).