Vulva, vagina & female urethra

Topic Completed: 1 February 2016

Minor changes: 13 November 2020

Copyright: 2002-2019,, Inc.

PubMed Search: Syringoma [title] vulva

Nicole Riddle, M.D.
Page views in 2020: 2,517
Page views in 2021 to date: 497
Cite this page: Riddle N. Syringoma. website. Accessed April 19th, 2021.
Definition / general
Essential features
  • Often multiple, 1 - 5 mm firm papules
  • Usually extragenital lesions as well
  • Well circumscribed dermal lesion with comma shaped ductules lined by basaloid type cells
  • Rare in vulva
  • Typically occurs after puberty; most common during reproductive years (may be due to hormonal influence)
  • More common in people of Asian decent
  • Increased incidence in Down, Nicolau-Balus and Brook-Spiegler syndromes
  • Possible familial association (up to 20%)
  • Eyelid > Cheek > Armpits > Abdomen > Vulva
  • Uncertain, possible hormonal influence
Clinical features
  • May be localized, generalized or eruptive (latter more common in Down Syndrome)
  • Multiple pruritic, sometimes painful papules
  • Often bilateral
  • Oral contraceptive pills or menstruation may worsen symptoms or increase lesion size
  • Commonly have concomitant extra-genital lesions as well
  • Symptoms may worsen in summer
Case reports
  • Treatment necessary only for cosmetic reasons or quality of life
  • Excision or cryotherapy / laser therapy is most common
  • Does not respond to topical steroids
Clinical images

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Facial and vulval syringomas

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Gross description
  • 1 - 5 mm firm skin colored papule with or without eruption
Microscopic (histologic) description
  • Well circumscribed proliferation of round to comma shaped ductules, nests, cysts and cords, lined by a double layer of basaloid-type cells with an eosinophilic cuticle
  • May have cell clearing glycogen (clear cell syringoma), more common in diabetics
  • Usually in superficial reticular dermis, rarely deep dermal extension
  • Stromal fibrosis
  • Variable luminal keratinaceous debris / milium material
  • No cytologic atypia
  • Very rare mitoses
Microscopic (histologic) images

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Contributed by Nicole Riddle, M.D.
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Superficial reticular dermis

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Basaloid-esque cells

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Severe pruritus

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Fibrosing stroma reaction

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Tumoral lesion

Cytology description
  • Basaloid cells, possibly in clusters
  • May be clear cells in clear cell variant
  • No cytologic atypia
  • Very rare mitoses
Positive stains
  • CK AE1/3
  • CEA: ductule cells
  • CK5, EMA: solid strands and outer cells of ductules
  • Some reports of PR, more than ER, however, more reports negative
Differential diagnosis
  • Desmoplastic trichoepithelioma: practically nonexistent in vulva, usually single lesion with keratinous cysts next to strands of basaloid cells and no eosinophilic cuticle
  • Microcystic adnexal carcinoma: practically nonexistent in vulva, poorly circumscribed, deeply infiltrative, with frequent perineural invasion
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