Zoon vulvitis

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Zoon vulvitis
Diagnosis in short

Zoon vulvitis. H&E stain.

Synonyms plasmacytosis mucosae

LM squamous epithelium with: spongiosis, lack of stratum granulosum and stratum corneum layers, "lozenge"-shaped (diamond-like shape); abundant plasma cells - esp. superficial dermis; +/-hemosiderin deposits
LM DDx syphilis, fungal infection
Stains PASF -ve
Site vulva

Symptoms pruritis, pain

Zoon vulvitis, also vulvitis circumscripta plasmacellularis[1] and plasma cell vulvitis, is a rare pathology of the vulva.

It is also known as plasmacytosis mucosae,[2] which may be used to refer to Zoon balanitis.

General

Clinical:[3]

  • Pruritis.
  • Pain.
  • Orange colouration.[5]

Gross

Features:[3]

  • Erythematous macules or papules.

Microscopic

Features:

  • Squamous epithelium with:[2]
    • Spongiosis.
    • Lack of stratum granulosum and stratum corneum layers.
    • "Lozenge"-shaped (diamond-like shape).
  • Abundant plasma cells - esp. superficial dermis.
    • Keep in mind that plasma cells are a common component of mucosal infiltrates, much more so than for skin.
    • A plasma cell component has to be relatively prominent to be relevant in this location (the same can be said for eosinophils).
  • Hemosiderin deposits.[6]

DDx:

  • Syphilis - Spirochete immunostain (in red) is helpful.
  • Spongiotic vulvitis.
    • Might look very similar.
    • Usually wont show iron deposition.
    • Clinical correlation important.
  • Superficial fungal vulvitis (usually Candida).
  • Lichenoid vulvitis (lichen planus/lichenoid drug reaction).
    • Both can be very plasmocellular on mucosal surfaces.
    • Zoon vulvitis won't show prominent lichenoid destruction of the epithelium.

Images

www:

IHC

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LESION, VULVA, BIOPSY:
- SQUAMOUS MUCOSA WITHOUT A CORNIFIED ZONE, WITH MARKED CHRONIC INFLAMMATION
  WITH PROMINENT PLASMA CELLS, SEE COMMENT.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

COMMENT:
The loss of the cornified zone and prominent plasma cells is consistent 
with plasma cell vulvitis (Zoon vulvitis). Clinical correlation is suggested.

A CD138 immunostain demonstrates clusters of plasma cells. A p16 immunostain 
is negative. Ki-67 positive cells are basal. No microorganisms are apparent 
with a PASF stain.  Perls stain positive iron is admixed with the inflammatory infiltrate.

See also

References

  1. dos Reis, HL.; de Vargas, PR.; Lucas, E.; Camporez, T.; Ferreira, Dde C.. "Zoon vulvitis as a differential diagnosis in an HIV-infected patient: a short report.". J Int Assoc Provid AIDS Care 12 (3): 159-61. doi:10.1177/2325957412467694. PMID 23449712.
  2. 2.0 2.1 Nucci, Marisa R.; Oliva, Esther (2009). Gynecologic Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 11. ISBN 978-0443069208.
  3. 3.0 3.1 3.2 3.3 Çelik, A.; Haliloglu, B.; Tanriöver, Y.; Ilter, E.; Gündüz, T.; Ulu, I.; Midi, A.; Özekici, Ü.. "Plasma cell vulvitis: a vulvar itching dilemma.". Indian J Dermatol Venereol Leprol 78 (2): 230. doi:10.4103/0378-6323.93664. PMID 22421677.
  4. Yoganathan, S.; Bohl, TG.; Mason, G. (Dec 1994). "Plasma cell balanitis and vulvitis (of Zoon). A study of 10 cases.". J Reprod Med 39 (12): 939-44. PMID 7884748.
  5. URL: http://www.dermnetnz.org/site-age-specific/plasma-cell.html. Accessed on: October 29, 2014.
  6. Davis, J.; Shapiro, L.; Baral, J. (Mar 1983). "Vulvitis circumscripta plasmacellularis.". J Am Acad Dermatol 8 (3): 413-6. PMID 6833541.