Sandbox:Zoon balanitis: Difference between revisions

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{{SK}}Balanoposthite chronique circonscrite bénigne á plasmocytes, Balanitis chronica circumscripta plasmacellularis
{{SK}}Balanoposthite chronique circonscrite bénigne á plasmocytes, Balanitis chronica circumscripta plasmacellularis, ZB, Zoon's vulvitis, Plasma cell vulvitis, Vulvitis circumscripta plasmacellularis
==Overview==
==Overview==
Zoon's balanitis is an idiopathic, chronic, benign inflammatory mucositis of the genitalia.  
Zoon's balanitis is a rare nonveneral idiopathic, chronic, benign inflammatory mucositis of genitalia. It was first described in the modern literature by Zoon, in 1952. The exact pathogenesis is not clearly known. It present as well circumscribed single or multiple, orange-red in colour with a characteristic glazed appearance and multiple pinpoint redder spots-"cayenne pepper spots" most commonly involving the glans penis.  


==Historical Perspective==
==Historical Perspective==
*In 1952, for the first time in medical literature, Zoon recognized a distinct entity in patients with chronic balanitis, named it as balanoposthite chronique circonscrite bénigne á plasmocytes” or “balanitis chronica circumscripta plasmacellularis.<ref name="pmid12979576">{{cite journal| author=ZOON JJ| title=[Chronic benign circumscript plasmocytic balanoposthitis]. | journal=Dermatologica | year= 1952 | volume= 105 | issue= 1 | pages= 1-7 | pmid=12979576 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12979576  }} </ref>
*In 1952, for the first time in medical literature, Zoon recognized a distinct entity in patients with chronic balanitis, named it as balanoposthite chronique circonscrite bénigne á plasmocytes” or “balanitis chronica circumscripta plasmacellularis.<ref name="pmid129795762">{{cite journal| author=ZOON JJ| title=[Chronic benign circumscript plasmocytic balanoposthitis]. | journal=Dermatologica | year= 1952 | volume= 105 | issue= 1 | pages= 1-7 | pmid=12979576 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12979576  }}</ref>
*In 1954, Garnier reported the similar lesion in vulva.<ref name="pmid7073984">{{cite journal| author=Sonnex TS, Dawber RP, Ryan TJ, Ralfs IG| title=Zoon's (plasma-cell) balanitis: treatment by circumcision. | journal=Br J Dermatol | year= 1982 | volume= 106 | issue= 5 | pages= 585-8 | pmid=7073984 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7073984  }} </ref>
*In 1954, Garnier reported the similar lesion in vulva.<ref name="pmid70739842">{{cite journal| author=Sonnex TS, Dawber RP, Ryan TJ, Ralfs IG| title=Zoon's (plasma-cell) balanitis: treatment by circumcision. | journal=Br J Dermatol | year= 1982 | volume= 106 | issue= 5 | pages= 585-8 | pmid=7073984 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7073984  }}</ref>
*In 1956, Nikolowski described the identical lesion in oral mucosa.<ref name="pmid13340789">{{cite journal| author=NIKOLOWSKI W, WIEHL R| title=[Not Available]. | journal=Arch Klin Exp Dermatol | year= 1956 | volume= 202 | issue= 4 | pages= 347-57 | pmid=13340789 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13340789  }} </ref>
*In 1956, Nikolowski described the identical lesion in oral mucosa.<ref name="pmid133407892">{{cite journal| author=NIKOLOWSKI W, WIEHL R| title=[Not Available]. | journal=Arch Klin Exp Dermatol | year= 1956 | volume= 202 | issue= 4 | pages= 347-57 | pmid=13340789 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13340789  }}</ref>
*In 1963, Kortnig described the idential lesion in conjuntiva.<ref name="pmid14098119">{{cite journal| author=KORTING GW, THEISEN H| title=[CIRCUMSCRIBED PLASMA CELL BALANOPOSTHITIS AND CONJUNCTIVITIS IN THE SAME PATIENT]. | journal=Arch Klin Exp Dermatol | year= 1963 | volume= 217 | issue=  | pages= 495-504 | pmid=14098119 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14098119  }} </ref>
*In 1963, Kortnig described the idential lesion in conjuntiva.<ref name="pmid140981192">{{cite journal| author=KORTING GW, THEISEN H| title=[CIRCUMSCRIBED PLASMA CELL BALANOPOSTHITIS AND CONJUNCTIVITIS IN THE SAME PATIENT]. | journal=Arch Klin Exp Dermatol | year= 1963 | volume= 217 | issue=  | pages= 495-504 | pmid=14098119 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14098119  }}</ref>
 
==Classification==
== Classification ==
There is no established classification system for Zoon balanitis.
There is no established classification system for Zoon balanitis.
==Pathophysiology==
==Pathophysiology==
===Pathogenesis===
===Pathogenesis===
The exact pathogenesis is not clearly known, but following theories have been postulated:<ref name="pmid11319970">{{cite journal| author=Porter WM, Bunker CB| title=The dysfunctional foreskin. | journal=Int J STD AIDS | year= 2001 | volume= 12 | issue= 4 | pages= 216-20 | pmid=11319970 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11319970  }}</ref>
The exact pathogenesis is not clearly known, but following theories have been postulated:<ref name="pmid113199702">{{cite journal| author=Porter WM, Bunker CB| title=The dysfunctional foreskin. | journal=Int J STD AIDS | year= 2001 | volume= 12 | issue= 4 | pages= 216-20 | pmid=11319970 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11319970  }}</ref>
* Accumlation of epithelial debris and secretions between foreskin and penis proximal to coronal sulcus, smegma,  poor genital hygiene, repeated local infections, hot and humid weather results in chronic physical irritation or subclinical trauma, which in turn results in skin lesion along the lines of the trauma.
*Accumlation of epithelial debris and secretions between foreskin and penis proximal to coronal sulcus, smegma,  poor genital hygiene, repeated local infections, hot and humid weather results in chronic physical irritation or subclinical trauma, which in turn results in skin lesion along the lines of the trauma.
* Chronic infection with ''Mycobacterium smegmatis'' and human papillomaviruses (HPV) was found to be associated with development of Zoon balanitis.<ref name="pmid15588560">{{cite journal| author=Pastar Z, Rados J, Lipozencić J, Skerlev M, Loncarić D| title=Zoon plasma cell balanitis: an overview and role of histopathology. | journal=Acta Dermatovenerol Croat | year= 2004 | volume= 12 | issue= 4 | pages= 268-73 | pmid=15588560 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15588560  }}</ref>
*Chronic infection with ''Mycobacterium smegmatis'' and human papillomaviruses (HPV) was found to be associated with development of Zoon balanitis.<ref name="pmid155885602">{{cite journal| author=Pastar Z, Rados J, Lipozencić J, Skerlev M, Loncarić D| title=Zoon plasma cell balanitis: an overview and role of histopathology. | journal=Acta Dermatovenerol Croat | year= 2004 | volume= 12 | issue= 4 | pages= 268-73 | pmid=15588560 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15588560  }}</ref>
* Many theories, which include 1) local disturbance of circulation, 2) hypersensitivity response mediated by IgE class of antibodies, 3) “extramedullary plasmacytic infiltrations that persists are expressions of occult multiple myeloma” have been postulated, no supportive evidence have been found for these hypothesis.<ref name="pmid12454596">{{cite journal| author=Weyers W, Ende Y, Schalla W, Diaz-Cascajo C| title=Balanitis of Zoon: a clinicopathologic study of 45 cases. | journal=Am J Dermatopathol | year= 2002 | volume= 24 | issue= 6 | pages= 459-67 | pmid=12454596 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12454596  }}</ref>
*Many theories, which include 1) local disturbance of circulation, 2) hypersensitivity response mediated by IgE class of antibodies, 3) “extramedullary plasmacytic infiltrations that persists are expressions of occult multiple myeloma” have been postulated, no supportive evidence have been found for these hypothesis.<ref name="pmid124545963">{{cite journal| author=Weyers W, Ende Y, Schalla W, Diaz-Cascajo C| title=Balanitis of Zoon: a clinicopathologic study of 45 cases. | journal=Am J Dermatopathol | year= 2002 | volume= 24 | issue= 6 | pages= 459-67 | pmid=12454596 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12454596  }}</ref>
 
====Histopathology====
==== Histopathology ====
ZB has distinctive histopathological features, which include:<ref name="pmid1245459622">{{cite journal| author=Weyers W, Ende Y, Schalla W, Diaz-Cascajo C| title=Balanitis of Zoon: a clinicopathologic study of 45 cases. | journal=Am J Dermatopathol | year= 2002 | volume= 24 | issue= 6 | pages= 459-67 | pmid=12454596 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12454596  }}</ref>
ZB has distinctive histopathological features, which include:
====Epidermal====
 
*Epidermal changes include, early thickening, acanthosis and parakeratosis of epidermis, which is followed by atrophy, erosions and spongiosis.
==== Epidermal ====
*Scattered neutrophils can be present in superficial erosions of the epidermis.
Epidermal changes include, early thickening, acanthosis and parakeratosis of epidermis, which is followed by atrophy, erosions and spongiosis. Scatth eutrophils can be present in super
*Spongiosis accentuation occurs in the lower half of the spinous zone.
 
*Subepidermal clefts, necrotic keratinocytes, and lozenge keratinocytes can be seen in the late stages of ZB.
Early changes
====Dermal====
 
*Dermal changes include  patchy lichenoid infiltrate of lymphocytes and plasma cells in papillary dermis, which are replaced by plasma cells, neutrophils, eosinophils, lymphocytes, and erythrocytes.
thickening, acanthosis, and parakeratosis, which is followed by followed by epidermal atrophy.
*Dermal  vascular dilatation with singular vertical or oblique orientation of proliferated individual vessels, is a characteristic feature  of ZB.
 
*In the later stages, upper dermis shows fibrosis which correlates well with subepidermal clefts, epidermal atrophy, and plasma cell infiltrates.
epidermal atrophy, can be associated with erosions and spongiosis.
 
Additional features include:
 
subepidermal clefts
 
necrotic keratinocytes,
 
lozenge keratinocytes
 
==== Dermal ====
ZB has very distinct histopathological changes affecting both epidermis and dermis.[13]
 
Epidermal changes → Earliest histopathological changes show epidermal thickening, acanthosis, and parakeratosis. This is followed by epidermal atrophy, at times erosions and spongiosis. These superficial erosions can be associated with scattered neutrophils in the upper reaches of epidermis. Accentuation of spongiosis occurs in the lower half of the spinous zone. Additional features such as subepidermal clefts, necrotic keratinocytes, and lozenge keratinocytes (i.e., elongated keratinocytes in the lower half of the spinous zone arranged parallel to the skin surface) may be seen in the later stages of ZB.
 
Dermal changes → Initially, there is a patchy lichenoid infiltrate of lymphocytes and some plasma cells in papillary dermis, which is subsequently replaced by dense band-like infiltrate of plasma cells, neutrophils, eosinophils, lymphocytes, and erythrocytes. Among all these infiltrate, plasmocytes are predominant usually exceeding 50% of all the cells. It may be associated with siderophages (i.e., macrophage that has absorbed iron-containing particles), hemosiderin deposition and extravasated red blood cells. Changes in dermal vasculature include vascular dilatation with singular vertical or oblique orientation of proliferated individual vessels, which is characteristic of ZB. These are more common in cases with a dense infiltrate. In the later stages, upper dermis shows fibrosis which correlates well with subepidermal clefts, epidermal atrophy, and plasma cell infiltrates. Immunohistochemical studies of plasmocytes in ZB show that plasmocytes produce immunoglobulin G (IgG) predominantly to a lesser degree IgA and IgM.[10]
 
It is relatively simple to differentiate the premalignant lesions from ZB histopathologically as one can see dysplastic epithelium in the premalignant lesions while it is absent in case of ZB. Histologically, benign conditions such as pemphigus vulgaris, flexural psoriasis, lichen planus, and Reiter's disease may show features in common with ZB but lack the typical changes in the epidermis and dermal blood vessels.
 
 
==Epidemiology and Demographics==
==Epidemiology and Demographics==
 
Epidemiological and Demographic data of Zoon balanitis is scare.
 
==Screening==
==Screening==
There is no established  screening guidelines for Zoon balanitis
There is no established  screening guidelines for Zoon balanitis
==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
 
===Natural history===
=== Natural history ===
If left untreated, there is risk for malignant transformation.<ref name="pmid27890945">{{cite journal| author=Dayal S, Sahu P| title=Zoon balanitis: A comprehensive review. | journal=Indian J Sex Transm Dis | year= 2016 | volume= 37 | issue= 2 | pages= 129-138 | pmid=27890945 | doi=10.4103/0253-7184.192128 | pmc=5111296 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27890945  }}</ref>


===Complications===
===Complications===
Complications of Zoon balanitis include:
* Phimosis
* Paraphimosis


===Prognosis===
===Prognosis===
Prognosis is good with treatment.<ref name="pmid278909452">{{cite journal| author=Dayal S, Sahu P| title=Zoon balanitis: A comprehensive review. | journal=Indian J Sex Transm Dis | year= 2016 | volume= 37 | issue= 2 | pages= 129-138 | pmid=27890945 | doi=10.4103/0253-7184.192128 | pmc=5111296 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27890945  }}</ref>


==Diagnosis==
==Diagnosis==
Diagnosis is made on by obtaining detailed history, physical examination and reflectance confocal microscopy/dermoscopy findings:
===History and symptoms===
===History and symptoms===
 
Patients with Zoon balanitits could be asymptomatic or present with:<ref name="pmid248285532">{{cite journal| author=Edwards SK, Bunker CB, Ziller F, van der Meijden WI| title=2013 European guideline for the management of balanoposthitis. | journal=Int J STD AIDS | year= 2014 | volume= 25 | issue= 9 | pages= 615-26 | pmid=24828553 | doi=10.1177/0956462414533099 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24828553  }}</ref>
*Itching (pruritus) of the genitalia.
*Discomfort in urination(dysuria)
*Pain in the gential region
*blood stain discharge
*Difficult or painful sexual intercourse
===Physical examination===
===Physical examination===
 
Physical examination findings include:<ref name="pmid155885602" /><ref name="pmid172415662">{{cite journal| author=Kumar B, Narang T, Dass Radotra B, Gupta S| title=Plasma cell balanitis: clinicopathologic study of 112 cases and treatment modalities. | journal=J Cutan Med Surg | year= 2006 | volume= 10 | issue= 1 | pages= 11-5 | pmid=17241566 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17241566  }}</ref>
* Well circumscribed single or multiple, orange-red in colour with a characteristic glazed appearance and multiple pinpoint redder spots-"cayenne pepper spots"(please [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5111296/figure/F2/ click here] to view the image) most commonly involving the glans penis, but inner surface of prepuce and coronal sulcus may be involved.
* Though uncommon, lesions of Zoon balanitis can involve other sites which include labia minora in females, oral mucosa, conjunctiva, urethra, cheeks, and epiglottis have been described in literature.<ref name="pmid24792459">{{cite journal| author=Adégbidi H, Atadokpèdé F, Dégboé B, Saka B, Akpadjan F, Yédomon H et al.| title=[Zoon's balanitis in circumcised and HIV infected man, at Cotonou (Benin)]. | journal=Bull Soc Pathol Exot | year= 2014 | volume= 107 | issue= 3 | pages= 139-41 | pmid=24792459 | doi=10.1007/s13149-014-0359-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24792459  }}</ref>
{| class="wikitable"
!Clinical criteria in diagnosing Zoon balanitis <ref name="pmid172415662" />
|-
|Shiny, erythematous patches on the glans, prepuce, or both
|-
|Lesion present for > 3months
|-
|Absence of lesion suggestive of Lichen planus, psoriasis elsewhere on the body
|-
|Poor response to topical therapies
|-
|Absence of concurrent infections which are ruled out after performing tzanck, potassium hydroxide, gram stain, and VDRL test.
|}
===Laboratory findings===
===Laboratory findings===
{| class="wikitable"
! colspan="2" |
|-
|Reflectance confocal microscopy
|A nucleated honeycomb pattern and vermicular vessels is a clue for benign inflammatory genital skin disease<ref name="pmid233254222">{{cite journal| author=Arzberger E, Komericki P, Ahlgrimm-Siess V, Massone C, Chubisov D, Hofmann-Wellenhof R| title=Differentiation between balanitis and carcinoma in situ using reflectance confocal microscopy. | journal=JAMA Dermatol | year= 2013 | volume= 149 | issue= 4 | pages= 440-5 | pmid=23325422 | doi=10.1001/jamadermatol.2013.2440 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23325422  }}</ref>
|-
|Dermoscopy
|Focal/diffuse orange-yellowish structure, less areas representing hemosiderin deposition, curved vessels due to epidermal thinning helps in distinguishing ZB from carcinoma in situ.<ref name="pmid266707162">{{cite journal| author=Errichetti E, Lacarrubba F, Micali G, Stinco G| title=Dermoscopy of Zoon's plasma cell balanitis. | journal=J Eur Acad Dermatol Venereol | year= 2016 | volume= 30 | issue= 12 | pages= e209-e210 | pmid=26670716 | doi=10.1111/jdv.13538 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26670716  }}</ref>
|-
|Biopsy
|Epidermis: Epidermis thickening which is followed by epidermal atrophy, at times with erosions.Dermis: Plasma cell infiltrate with haemosiderin and extravasated red blood cells.
|}
==Treatment==


==Treatment==
=== General measures ===
Good hygiene which include retracting the foreskin regularly and gentle cleansing of entire glans, preputial sac, and foreskin were found effective in treating the diseases.<ref name="pmid24828553">{{cite journal| author=Edwards SK, Bunker CB, Ziller F, van der Meijden WI| title=2013 European guideline for the management of balanoposthitis. | journal=Int J STD AIDS | year= 2014 | volume= 25 | issue= 9 | pages= 615-26 | pmid=24828553 | doi=10.1177/0956462414533099 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24828553  }}</ref>


===Medical Therapy===
===Medical Therapy===
{| class="wikitable"
! colspan="3" |Various medical managements for Zoon's balanitis
!
|-
!
!Drug dosage
!Effectiveness
!
|-
| rowspan="2" |Topical steroids
|Saline compresses containing 1% hydrocortisone/0.02% betamethason+/-17-valerate/0.05% betamethasone dipropionate
|3 out of 6 patients responded
|
|-
|Oxytetracycline 3%, nystatin 100,00(units/g), and clobetasone butyrate 0.05% applied until complete resolution was observed
|All patients responded, but 3 out of 10 patients had recurrences
|
|-
|Topical calineurin
|Tacrolimus ointment 0.1% twice daily
|Complete remission after 4 weeks of treatment was observed in 9 patients , with no relapse observed after 3 months of follow up
|
|-
|Topical Pimecrolimus
|Pimecrolimus cream 1% twice daily
|Improvement is observed after 2 months of treatment with no relapse observed
|
|-
| rowspan="2" |Topical Imiquimod
|5% imiquimod cream, 3 times a week for 4 months with multiple periods without treatment
| rowspan="2" |Complete resolution can be found after 4-12 weeks of treatment, with no cases of relapse observed
|
|-
|5% imiquimod cream, 3 times a week for 12 months without any interruption
|
|}


=== Surgery ===
{| class="wikitable"
! colspan="2" |Various surgical modalities for Zoon's balanitis
|-
|Circumcision
|Lesion disappear by 5-6 weeks after procedure, with no relapse  observed 
|-
|Carbon dioxide lesion
|Complete resolution in 3 months,  with no relapse observed in following 5 years of follow up
|-
|Yag laser
|Complete clearance is seen patients within 2-3 weeks, with no relapse observed in following 30 months of follow up
|-
|PDT
|Lesion healed completely after an average 2.75 PDT sessions, with no relapse observed in following 1 year of follow up
|}


=== Photodynamic therapy ===
Photodynamic therapy, 5-aminolaevulinic acid (ALA) or methyl aminolevulinate (MAL), has been proposed for refractory lesions of ZB. ALA-PDT seems to be slightly better than MAL-PDT, with no long-term side effects observed.<ref name="pmid22693017">{{cite journal| author=Pinto-Almeida T, Vilaça S, Amorim I, Costa V, Alves R, Selores M| title=Complete resolution of Zoon balanitis with photodynamic therapy--a new therapeutic option? | journal=Eur J Dermatol | year= 2012 | volume= 22 | issue= 4 | pages= 540-1 | pmid=22693017 | doi=10.1684/ejd.2012.1779 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22693017  }}</ref>
=== Miscellaneous therapies ===
Once-daily application fusidic acid cream for 8-16 weeks was effective in suppression and cure of ZB.<ref name="pmid1401323">{{cite journal| author=Petersen CS, Thomsen K| title=Fusidic acid cream in the treatment of plasma cell balanitis. | journal=J Am Acad Dermatol | year= 1992 | volume= 27 | issue= 4 | pages= 633-4 | pmid=1401323 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1401323  }}</ref>


==Prevention==
==Prevention==
 
===Primary Prevention===
=== Primary Prevention ===
Circumcision in males can help in reducing risk of having ZB.<ref name="pmid278909453">{{cite journal| author=Dayal S, Sahu P| title=Zoon balanitis: A comprehensive review. | journal=Indian J Sex Transm Dis | year= 2016 | volume= 37 | issue= 2 | pages= 129-138 | pmid=27890945 | doi=10.4103/0253-7184.192128 | pmc=5111296 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27890945  }}</ref>


===Secondary prevention===
===Secondary prevention===
There is no secondary prevention measures.


==References==
==References==

Latest revision as of 17:35, 8 February 2017


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

Synonyms and keywords:Balanoposthite chronique circonscrite bénigne á plasmocytes, Balanitis chronica circumscripta plasmacellularis, ZB, Zoon's vulvitis, Plasma cell vulvitis, Vulvitis circumscripta plasmacellularis

Overview

Zoon's balanitis is a rare nonveneral idiopathic, chronic, benign inflammatory mucositis of genitalia. It was first described in the modern literature by Zoon, in 1952. The exact pathogenesis is not clearly known. It present as well circumscribed single or multiple, orange-red in colour with a characteristic glazed appearance and multiple pinpoint redder spots-"cayenne pepper spots" most commonly involving the glans penis.

Historical Perspective

  • In 1952, for the first time in medical literature, Zoon recognized a distinct entity in patients with chronic balanitis, named it as balanoposthite chronique circonscrite bénigne á plasmocytes” or “balanitis chronica circumscripta plasmacellularis.[1]
  • In 1954, Garnier reported the similar lesion in vulva.[2]
  • In 1956, Nikolowski described the identical lesion in oral mucosa.[3]
  • In 1963, Kortnig described the idential lesion in conjuntiva.[4]

Classification

There is no established classification system for Zoon balanitis.

Pathophysiology

Pathogenesis

The exact pathogenesis is not clearly known, but following theories have been postulated:[5]

  • Accumlation of epithelial debris and secretions between foreskin and penis proximal to coronal sulcus, smegma, poor genital hygiene, repeated local infections, hot and humid weather results in chronic physical irritation or subclinical trauma, which in turn results in skin lesion along the lines of the trauma.
  • Chronic infection with Mycobacterium smegmatis and human papillomaviruses (HPV) was found to be associated with development of Zoon balanitis.[6]
  • Many theories, which include 1) local disturbance of circulation, 2) hypersensitivity response mediated by IgE class of antibodies, 3) “extramedullary plasmacytic infiltrations that persists are expressions of occult multiple myeloma” have been postulated, no supportive evidence have been found for these hypothesis.[7]

Histopathology

ZB has distinctive histopathological features, which include:[8]

Epidermal

  • Epidermal changes include, early thickening, acanthosis and parakeratosis of epidermis, which is followed by atrophy, erosions and spongiosis.
  • Scattered neutrophils can be present in superficial erosions of the epidermis.
  • Spongiosis accentuation occurs in the lower half of the spinous zone.
  • Subepidermal clefts, necrotic keratinocytes, and lozenge keratinocytes can be seen in the late stages of ZB.

Dermal

  • Dermal changes include patchy lichenoid infiltrate of lymphocytes and plasma cells in papillary dermis, which are replaced by plasma cells, neutrophils, eosinophils, lymphocytes, and erythrocytes.
  • Dermal vascular dilatation with singular vertical or oblique orientation of proliferated individual vessels, is a characteristic feature of ZB.
  • In the later stages, upper dermis shows fibrosis which correlates well with subepidermal clefts, epidermal atrophy, and plasma cell infiltrates.

Epidemiology and Demographics

Epidemiological and Demographic data of Zoon balanitis is scare.

Screening

There is no established screening guidelines for Zoon balanitis

Natural History, Complications, and Prognosis

Natural history

If left untreated, there is risk for malignant transformation.[9]

Complications

Complications of Zoon balanitis include:

  • Phimosis
  • Paraphimosis

Prognosis

Prognosis is good with treatment.[10]

Diagnosis

Diagnosis is made on by obtaining detailed history, physical examination and reflectance confocal microscopy/dermoscopy findings:

History and symptoms

Patients with Zoon balanitits could be asymptomatic or present with:[11]

  • Itching (pruritus) of the genitalia.
  • Discomfort in urination(dysuria)
  • Pain in the gential region
  • blood stain discharge
  • Difficult or painful sexual intercourse

Physical examination

Physical examination findings include:[6][12]

  • Well circumscribed single or multiple, orange-red in colour with a characteristic glazed appearance and multiple pinpoint redder spots-"cayenne pepper spots"(please click here to view the image) most commonly involving the glans penis, but inner surface of prepuce and coronal sulcus may be involved.
  • Though uncommon, lesions of Zoon balanitis can involve other sites which include labia minora in females, oral mucosa, conjunctiva, urethra, cheeks, and epiglottis have been described in literature.[13]
Clinical criteria in diagnosing Zoon balanitis [12]
Shiny, erythematous patches on the glans, prepuce, or both
Lesion present for > 3months
Absence of lesion suggestive of Lichen planus, psoriasis elsewhere on the body
Poor response to topical therapies
Absence of concurrent infections which are ruled out after performing tzanck, potassium hydroxide, gram stain, and VDRL test.

Laboratory findings

Reflectance confocal microscopy A nucleated honeycomb pattern and vermicular vessels is a clue for benign inflammatory genital skin disease[14]
Dermoscopy Focal/diffuse orange-yellowish structure, less areas representing hemosiderin deposition, curved vessels due to epidermal thinning helps in distinguishing ZB from carcinoma in situ.[15]
Biopsy Epidermis: Epidermis thickening which is followed by epidermal atrophy, at times with erosions.Dermis: Plasma cell infiltrate with haemosiderin and extravasated red blood cells.

Treatment

General measures

Good hygiene which include retracting the foreskin regularly and gentle cleansing of entire glans, preputial sac, and foreskin were found effective in treating the diseases.[16]

Medical Therapy

Various medical managements for Zoon's balanitis
Drug dosage Effectiveness
Topical steroids Saline compresses containing 1% hydrocortisone/0.02% betamethason+/-17-valerate/0.05% betamethasone dipropionate 3 out of 6 patients responded
Oxytetracycline 3%, nystatin 100,00(units/g), and clobetasone butyrate 0.05% applied until complete resolution was observed All patients responded, but 3 out of 10 patients had recurrences
Topical calineurin Tacrolimus ointment 0.1% twice daily Complete remission after 4 weeks of treatment was observed in 9 patients , with no relapse observed after 3 months of follow up
Topical Pimecrolimus Pimecrolimus cream 1% twice daily Improvement is observed after 2 months of treatment with no relapse observed
Topical Imiquimod 5% imiquimod cream, 3 times a week for 4 months with multiple periods without treatment Complete resolution can be found after 4-12 weeks of treatment, with no cases of relapse observed
5% imiquimod cream, 3 times a week for 12 months without any interruption

Surgery

Various surgical modalities for Zoon's balanitis
Circumcision Lesion disappear by 5-6 weeks after procedure, with no relapse observed
Carbon dioxide lesion Complete resolution in 3 months, with no relapse observed in following 5 years of follow up
Yag laser Complete clearance is seen patients within 2-3 weeks, with no relapse observed in following 30 months of follow up
PDT Lesion healed completely after an average 2.75 PDT sessions, with no relapse observed in following 1 year of follow up

Photodynamic therapy

Photodynamic therapy, 5-aminolaevulinic acid (ALA) or methyl aminolevulinate (MAL), has been proposed for refractory lesions of ZB. ALA-PDT seems to be slightly better than MAL-PDT, with no long-term side effects observed.[17]

Miscellaneous therapies

Once-daily application fusidic acid cream for 8-16 weeks was effective in suppression and cure of ZB.[18]

Prevention

Primary Prevention

Circumcision in males can help in reducing risk of having ZB.[19]

Secondary prevention

There is no secondary prevention measures.

References

  1. ↑ ZOON JJ (1952). "[Chronic benign circumscript plasmocytic balanoposthitis]". Dermatologica. 105 (1): 1–7. PMID 12979576.
  2. ↑ Sonnex TS, Dawber RP, Ryan TJ, Ralfs IG (1982). "Zoon's (plasma-cell) balanitis: treatment by circumcision". Br J Dermatol. 106 (5): 585–8. PMID 7073984.
  3. ↑ NIKOLOWSKI W, WIEHL R (1956). "[Not Available]". Arch Klin Exp Dermatol. 202 (4): 347–57. PMID 13340789.
  4. ↑ KORTING GW, THEISEN H (1963). "[CIRCUMSCRIBED PLASMA CELL BALANOPOSTHITIS AND CONJUNCTIVITIS IN THE SAME PATIENT]". Arch Klin Exp Dermatol. 217: 495–504. PMID 14098119.
  5. ↑ Porter WM, Bunker CB (2001). "The dysfunctional foreskin". Int J STD AIDS. 12 (4): 216–20. PMID 11319970.
  6. ↑ 6.0 6.1 Pastar Z, Rados J, Lipozencić J, Skerlev M, Loncarić D (2004). "Zoon plasma cell balanitis: an overview and role of histopathology". Acta Dermatovenerol Croat. 12 (4): 268–73. PMID 15588560.
  7. ↑ Weyers W, Ende Y, Schalla W, Diaz-Cascajo C (2002). "Balanitis of Zoon: a clinicopathologic study of 45 cases". Am J Dermatopathol. 24 (6): 459–67. PMID 12454596.
  8. ↑ Weyers W, Ende Y, Schalla W, Diaz-Cascajo C (2002). "Balanitis of Zoon: a clinicopathologic study of 45 cases". Am J Dermatopathol. 24 (6): 459–67. PMID 12454596.
  9. ↑ Dayal S, Sahu P (2016). "Zoon balanitis: A comprehensive review". Indian J Sex Transm Dis. 37 (2): 129–138. doi:10.4103/0253-7184.192128. PMC 5111296. PMID 27890945.
  10. ↑ Dayal S, Sahu P (2016). "Zoon balanitis: A comprehensive review". Indian J Sex Transm Dis. 37 (2): 129–138. doi:10.4103/0253-7184.192128. PMC 5111296. PMID 27890945.
  11. ↑ Edwards SK, Bunker CB, Ziller F, van der Meijden WI (2014). "2013 European guideline for the management of balanoposthitis". Int J STD AIDS. 25 (9): 615–26. doi:10.1177/0956462414533099. PMID 24828553.
  12. ↑ 12.0 12.1 Kumar B, Narang T, Dass Radotra B, Gupta S (2006). "Plasma cell balanitis: clinicopathologic study of 112 cases and treatment modalities". J Cutan Med Surg. 10 (1): 11–5. PMID 17241566.
  13. ↑ Adégbidi H, Atadokpèdé F, Dégboé B, Saka B, Akpadjan F, Yédomon H; et al. (2014). "[Zoon's balanitis in circumcised and HIV infected man, at Cotonou (Benin)]". Bull Soc Pathol Exot. 107 (3): 139–41. doi:10.1007/s13149-014-0359-4. PMID 24792459.
  14. ↑ Arzberger E, Komericki P, Ahlgrimm-Siess V, Massone C, Chubisov D, Hofmann-Wellenhof R (2013). "Differentiation between balanitis and carcinoma in situ using reflectance confocal microscopy". JAMA Dermatol. 149 (4): 440–5. doi:10.1001/jamadermatol.2013.2440. PMID 23325422.
  15. ↑ Errichetti E, Lacarrubba F, Micali G, Stinco G (2016). "Dermoscopy of Zoon's plasma cell balanitis". J Eur Acad Dermatol Venereol. 30 (12): e209–e210. doi:10.1111/jdv.13538. PMID 26670716.
  16. ↑ Edwards SK, Bunker CB, Ziller F, van der Meijden WI (2014). "2013 European guideline for the management of balanoposthitis". Int J STD AIDS. 25 (9): 615–26. doi:10.1177/0956462414533099. PMID 24828553.
  17. ↑ Pinto-Almeida T, Vilaça S, Amorim I, Costa V, Alves R, Selores M (2012). "Complete resolution of Zoon balanitis with photodynamic therapy--a new therapeutic option?". Eur J Dermatol. 22 (4): 540–1. doi:10.1684/ejd.2012.1779. PMID 22693017.
  18. ↑ Petersen CS, Thomsen K (1992). "Fusidic acid cream in the treatment of plasma cell balanitis". J Am Acad Dermatol. 27 (4): 633–4. PMID 1401323.
  19. ↑ Dayal S, Sahu P (2016). "Zoon balanitis: A comprehensive review". Indian J Sex Transm Dis. 37 (2): 129–138. doi:10.4103/0253-7184.192128. PMC 5111296. PMID 27890945.

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