Zoon balanitis
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] |
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 Zoons 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
- ↑ ZOON JJ (1952). "[Chronic benign circumscript plasmocytic balanoposthitis]". Dermatologica. 105 (1): 1–7. PMID 12979576.
- ↑ 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.
- ↑ NIKOLOWSKI W, WIEHL R (1956). "[Not Available]". Arch Klin Exp Dermatol. 202 (4): 347–57. PMID 13340789.
- ↑ KORTING GW, THEISEN H (1963). "[CIRCUMSCRIBED PLASMA CELL BALANOPOSTHITIS AND CONJUNCTIVITIS IN THE SAME PATIENT]". Arch Klin Exp Dermatol. 217: 495–504. PMID 14098119.
- ↑ Porter WM, Bunker CB (2001). "The dysfunctional foreskin". Int J STD AIDS. 12 (4): 216–20. PMID 11319970.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 12.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.