2010-09-14 追访临床补充病史

考虑外阴白色角化病

 谢谢wy1992老师提供的思路。  

Review for Extramammary Paget's Disease

Extramammary Paget’s disease (EMPD), also Extramammary Paget disease, is a rare, slow-growing, usually non-invasive intraepithelial (in the skin) adenocarcinoma outside of the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.

Extramammary Paget’s disease involves primarily the epidermis but occasionally extends into the underlying dermis.It has a predilection for apocrine gland bearing areas, mostly the perineum, vulva, axilla, scrotum, and penis.

Prognosis and comorbity
Extramammary Paget's disease is usually seen in isolation and is not usually associated with an underlying invasive malignancy. This is unlike Paget's disease of the breast, which is almost always associated with an underlying invasive malignancy, i.e. breast cancer (e.g. mammary ductal carcinoma).

Signs and symptoms
Signs and symptoms are a skin lesion often mistaken as eczema that may be itchy or painful. A biopsy will establish the diagnosis. The histology of the lesion is the same as for Paget's disease of the breast.

Types
Paget's disease of the vulva, a rare disease, may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra, or the rectum and thus be secondary. Patients tend to be postmenopausal.

Paget's disease of the penis may also be primary or secondary and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease.Isolated Paget's disease of the penis is extremely rare.

Primary disease
It is important to exclude that the lesion is associated with another cancer. Primary disease is usually treated by surgical excision.

History
James Paget described Paget's disease of the nipple in 1874. Radcliffe Crocker reported the first case of EMPD in 1889 when he described a patient with a skin lesion affecting the penis and scrotum, the findings of which were identical to those described by Paget.

Micrograph of extramammary Paget's disease. H&E stain.

名称：图1
描述：图1

  wfbjwt

Review for Bowen's Disease

Bowen's disease (BD) (also known as "squamous cell carcinoma in situ"[1]:655) is a neoplastic skin disease, it can be considered as an early stage or intraepidermal form of squamous cell carcinoma. It was named after Dr John T. Bowen, the doctor who first described it in 1912. Erythroplasia of Queyrat is a form of squamous cell carcinoma in situ arising on the glans or prepuce, possibly induced by HPV.[2]

Causes
Causes of BD include solar damage, arsenic, immunosuppression (including AIDS), viral infection (human papillomavirus or HPV) and chronic skin injury and dermatoses.

Signs and symptoms
Bowen's disease typically presents as a gradually enlarging, well demarcated erythematous plaque with an irregular border and surface crusting or scaling. BD may occur at any age in adults but is rare before the age of 30 years - most patients are aged over 60. Any site may be affected, although involvement of palms or soles is uncommon. BD occurs predominantly in women (70-85% of cases). About 60-85% of patients have lesions on the lower leg, usually in previously or presently sun exposed areas of skin.

This is a persistent progressive non-elevated red scaly or crusted plaque which is due to an intraepidermal carcinoma and is potentially malignant. The lesions may occur anywhere on the skin surface or on mucosal surfaces. Freezing, cauterization or diathermy coagulation is often effective treatment.

Histology
Bowen's disease is essentially equivalent to squamous cell carcinoma in situ. Atypical squamous cells proliferate through the whole thickness of the epidermis. The entire tumor is confined to the epidermis and does not invade into the dermis. The cells in Bowen's are often highly atypical under the microscope, and may in fact look more unusual than the cells of some invasive squamous cell carcinomas.

Additional images

onmouseover="showMenu(this.id, 0, 1)">

几例Vulvar Intraepithelial Neoplasia VIN 临床表现外观图像

同意鲍温病，染pas看看，进一步除外派杰病。谢谢Rebecca老师分享大量病例图片和文献！

Rewiew for Vulvar Intraepithelial Neoplasia VIN

The term Vulvar intraepithelial neoplasia (VIN) refers to particular changes that can occur in the skin that covers the vulva. VIN is not cancer, and in some women it disappears without treatment. If the changes become more severe, there is a chance that cancer might develop after many years, and so it is referred to as a precancerous condition.


ISSVD Classification
Medically speaking, the term denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential.

The terminology of VIN evolved over several decades. In 1989[2] the Committee on Terminology, International Society for the Study of Vulvar Disease (ISSVD) replaced older terminology such as vulvar dystrophy, Bowen's disease, and Kraurosis vulvae by a new classification system for Epithelial Vulvar Disease:

Nonneoplastic epithelial disorders of vulva and mucosa:
   Lichen sclerosus
   Squamous hyperplasia
   Other dermatoses
Mixed neoplastic and nonneoplastic disorders
Intraepithelial neoplasia

Squamous vulvar intraepithelial neoplasia (VIN)
   VIN I, mildest form
   VIN II, intermediate
   VIN III, most severe form including carcinoma in situ of the vulva

Non-squamous intraepithelial neoplasia
   Extramammary Paget's disease
   Tumors of melanocytes, non invasive
Invasive disease (vulvar carcinoma)

Diagnosis
The patient may have no symptoms, or local symptomatology including itching, burning, and pain. The diagnosis is always based on a careful inspection and a targeted biopsy.

Treatment
The treatment of VIN is local to wide excision, in case of very extensive involvement or recurrency even a simple vulvectomy. Laser therapy has also been useful for VIN.

Additional images:

Micrograph of vulvar intraepithelial neoplasia III or Grade 3 (VIN 3). H&E stain.

名称：图1
描述：图1

名称：图2
描述：图2

名称：图3
描述：图3