Difference between revisions of "Zoon vulvitis"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =  Plasmacytosis mucosae -- high mag.jpg
| Width      =
| Caption    = Zoon vulvitis. [[H&E stain]].
| Synonyms  = [[plasmacytosis mucosae]]
| Micro      = 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
| Subtypes  =
| LMDDx      = [[syphilis]], fungal infection
| Stains    = PASF -ve
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[vulva]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = pruritis, pain
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Zoon vulvitis''', also '''vulvitis circumscripta plasmacellularis'''<ref>{{Cite journal  | last1 = dos Reis | first1 = HL. | last2 = de Vargas | first2 = PR. | last3 = Lucas | first3 = E. | last4 = Camporez | first4 = T. | last5 = Ferreira | first5 = Dde C. | title = Zoon vulvitis as a differential diagnosis in an HIV-infected patient: a short report. | journal = J Int Assoc Provid AIDS Care | volume = 12 | issue = 3 | pages = 159-61 | month =  | year =  | doi = 10.1177/2325957412467694 | PMID = 23449712 }}</ref> and '''plasma cell vulvitis''', is a rare pathology of the [[vulva]].
'''Zoon vulvitis''', also '''vulvitis circumscripta plasmacellularis'''<ref>{{Cite journal  | last1 = dos Reis | first1 = HL. | last2 = de Vargas | first2 = PR. | last3 = Lucas | first3 = E. | last4 = Camporez | first4 = T. | last5 = Ferreira | first5 = Dde C. | title = Zoon vulvitis as a differential diagnosis in an HIV-infected patient: a short report. | journal = J Int Assoc Provid AIDS Care | volume = 12 | issue = 3 | pages = 159-61 | month =  | year =  | doi = 10.1177/2325957412467694 | PMID = 23449712 }}</ref> and '''plasma cell vulvitis''', is a rare pathology of the [[vulva]].
It is also known as ''[[plasmacytosis mucosae]]'',<ref name=Ref_GP11>{{Ref GP|11}}</ref> which may be used to refer to ''[[Zoon balanitis]]''.


==General==
==General==
*Rare.
*Rare ~ 50 reported cases in English literature as of 2012.<ref name=pmid22421677>{{Cite journal  | last1 = Çelik | first1 = A. | last2 = Haliloglu | first2 = B. | last3 = Tanriöver | first3 = Y. | last4 = Ilter | first4 = E. | last5 = Gündüz | first5 = T. | last6 = Ulu | first6 = I. | last7 = Midi | first7 = A. | last8 = Özekici | first8 = Ü. | title = Plasma cell vulvitis: a vulvar itching dilemma. | journal = Indian J Dermatol Venereol Leprol | volume = 78 | issue = 2 | pages = 230 | month =  | year =  | doi = 10.4103/0378-6323.93664 | PMID = 22421677 | URL = http://www.ijdvl.com/article.asp?issn=0378-6323;year=2012;volume=78;issue=2;spage=230;epage=230;aulast=%C7elik }}</ref>
*Analogous to [[Zoon's balanitis]] in males.
*Analogous to [[Zoon's balanitis]] in males.<ref name=pmid7884748>{{Cite journal  | last1 = Yoganathan | first1 = S. | last2 = Bohl | first2 = TG. | last3 = Mason | first3 = G. | title = Plasma cell balanitis and vulvitis (of Zoon). A study of 10 cases. | journal = J Reprod Med | volume = 39 | issue = 12 | pages = 939-44 | month = Dec | year = 1994 | doi =  | PMID = 7884748 }}</ref>
 
Clinical:<ref name=pmid22421677/>
*Pruritis.
*Pain.
*Orange colouration.<ref>URL: [http://www.dermnetnz.org/site-age-specific/plasma-cell.html http://www.dermnetnz.org/site-age-specific/plasma-cell.html]. Accessed on: October 29, 2014.</ref>
 
==Gross==
Features:<ref name=pmid22421677/>
*Erythematous macules or papules.


==Microscopic==
==Microscopic==
Features:
Features:
*Lack of a cornified layer.
*Squamous epithelium with:<ref name=Ref_GP11>{{Ref GP|11}}</ref>
*Abundant plasma cells.
**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.<ref name=pmid6833541>{{Cite journal  | last1 = Davis | first1 = J. | last2 = Shapiro | first2 = L. | last3 = Baral | first3 = J. | title = Vulvitis circumscripta plasmacellularis. | journal = J Am Acad Dermatol | volume = 8 | issue = 3 | pages = 413-6 | month = Mar | year = 1983 | doi =  | PMID = 6833541 }}</ref>


DDx:
DDx:
*[[Syphilis]].
*[[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====
<gallery>
Image: Plasmacytosis mucosae -- intermed mag.jpg | PM - intermed. mag.
Image: Plasmacytosis mucosae -- high mag.jpg | PM - high mag.
Image: Plasmacytosis mucosae - alt -- high mag.jpg | PM - high mag.
Image: Plasmacytosis mucosae -- very high mag.jpg | PM - very high mag.
</gallery>
www:
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?s=20080802172103984 Vulvitis chronica plasmacellularis (surgicalpathologyatlas.com)].
*[http://www.ijdvl.com/viewimage.asp?img=ijdvl_2012_78_2_230_93664_f2.jpg Zoon vulvitis (ijdvl.com)].<ref name=pmid22421677/>
 
==IHC==
*CD138 -- to highlight [[plasma cell]]s.
 
==Sign out==
<pre>
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.
</pre>


==See also==
==See also==
*[[Vulva]].
*[[Vulva]].
*[[Zoon balanitis]].


==References==
==References==

Latest revision as of 03:43, 14 November 2014

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

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

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

  • PM - intermed. mag.

  • PM - high mag.

  • PM - high mag.

  • PM - very high mag.

www:

IHC

Sign out

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.
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