Herpes Vulvitis

22/02/53
* 1 st described in 1888, Crocker reported Paget
disease of scrotum and penis, 2 o to bladder CA
* Most commonly involves external genitalia, less
common over perianus
* Also reported in other areas of axilla, umbilicus,
groins, eyelids, external ear canal.
* Age: 45-91 yrs (median 67 yrs)
* Associated carcinoma: 4-20%
* Surgery: radical vulvectomy, partial vulvectomy,
wide local l excision
i
* Recurrence rate 30-69% after surgery
* Free margin : F.S., fluorescein-aided visualization
(Controversy on significance of surgical margin)
Wilkinson EJ & Brown HB, Human Pathology. 2003
Brummer et al. Gynecol Oncol 2004;95:336-40.
Molinie etal. Ann Dermatol Venereol 1993;120:522-7
Lu et al. Zhonghua Fu Chan Ke Za Zhi 1999;34:156-8
Misas et al. Obstet Gynecol 1991;77:156-9.
Primary Paget disease:
* as a 1 o intraepithelial neoplasm.
* as an intraepithelial neoplasm with invasion.
* as a manifestation of an underlying cutaneous, or vulvar ACA
Secondary Paget disease:
* 2 o to adjacent non-cutaneous ACA (e.g. anal or rectal ACA)
* of urothelial origin (PUIN) (pagetoid urothelial intraepithelial neoplasia)
PUIN as a manifestation of intraepithelial urothelial neoplasia (CIS)
or invasive urothelial CA
* of other origins
Wilkinson EJ and Brown HB, Human Pathology. 2003
1 o lesion (more common)
* arise within epidermis & extend into
contiguous epithelium of skin
* arise from skin appendages (usually
apocrine glands) & extend to overlying
epidermis by epidermotropism.
2 o lesion
* non-cutaneous CA--- involves skin by direct
extension or epidermotropic metastasis
Wilkinson EJ and Brown HB, Human Pathology. 2003
Paget disease in
vulvectomy specimen
Paget disease of vulva
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