Zoon vulvitis

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

General

Clinical:[2]

  • Pruritis.
  • Pain.

Gross

Features:[2]

  • Erythematous macules or papules.

Microscopic

Features:

  • Squamous epithelium with:[4]
    • Spongiosis.
    • Lack of stratum granulosum and stratum corneum layers.
    • "Lozenge"-shaped (diamond-like shape).
  • Abundant plasma cells - esp. superficial dermis.
  • Hemosiderin deposits.[5]

DDx:

Images

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.

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 2.2 2.3 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.
  3. 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.
  4. 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.
  5. Davis, J.; Shapiro, L.; Baral, J. (Mar 1983). "Vulvitis circumscripta plasmacellularis.". J Am Acad Dermatol 8 (3): 413-6. PMID 6833541.