Pathophysiology
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• Strongest clinical association is with obesity (Int J Gynecol Pathol 2011;30:306)
• Likely due to lymphatic obstruction with lymphedema (J Cutan Pathol 2009;36:1)

Clinical features
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• Patient age range 19-59 years
• Present with papillomatous plaques, skin polyps, generalized vulvar enlargement, or massive pedunculated masses that had been present for variable durations

Case reports
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• 19 and 59 year old morbidly obese women (Int J Gynecol Pathol 2011;30:306)
• 43 year old woman (Arch Pathol Lab Med 2000;124:1697)
• 2 patients, one overweight (Gynecol Obstet Invest 2010;69:212)

Treatment
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• A simple surgical excision with vulvoplasty is curative

Micro description
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• Stromal edema with hypocellular proliferation of fibroblastic cells, vessels with perivascular collagen deposition, ectatic tortuous lymphatics
• No true thick-walled vessels
• Other possible findings include multinucleated giant cells, dermal fibrosis, dermal chronic inflammation, perivascular chronic inflammation (superficial or deep), hyperkeratosis, acanthosis, and blood vessels of varying calibers

Micro images
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Fibroblastic cells in edematous myxoid stroma

Blood vessels with perivascular collagen deposition

Dilated and tortuous lymphatics

Edematous and collagenous dermis

Differential diagnosis
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• Aggressive angiomyxoma

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