Zoon's 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 non-veneral idiopathic, chronic, benign inflammatory mucositis of genitalia. In 1952, for the first time in medical literature, Zoon recognized a distinct entity in patients with chronic balanitis, named it has balanoposthite chronique circonscrite bénigne á plasmocytes” or “balanitis chronica circumscripta plasmacellularis.[1] The exact pathogenesis is not clearly known. Patients with Zoon's balanitits present with 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 glans penis. Diagnosis of Zoon's balanitis is confirmed by biopsy. Management of Zoon's balanitis includes both medical and surgical modalities.
Historical Perspective
- In 1952, for the first time in medical literature, Zoon recognized a distinct entity in patients with chronic balanitis, named it has balanoposthite chronique circonscrite bénigne á plasmocytes” or “balanitis chronica circumscripta plasmacellularis.[1]
- In 1954, Garnier reported the similar lesions in vulva.[2]
- In 1956, Nikolowski described the identical lesions in oral mucosa.[3]
- In 1963, Kortnig described the idential lesions in conjunctiva.[4]
Classification
There is no established classification system for Zoon's balanitis.
Pathophysiology
Pathogenesis
The exact pathogenesis of Zoon's balanitis is not clearly known, but following theories have been postulated:[5]
- Accumulation of epithelial debris and secretions between foreskin and penis proximal to coronal sulcus, smegma, poor genital hygiene, repeated local infections, and hot and humid weather results in chronic physical irritation or sub clinical trauma. Chronic physical irritation or sub-clinical trauma in-turn results in skin lesions along the line of trauma.
- Chronic infection with Mycobacterium smegmatis and human papillomaviruses (HPV) was found to be associated with development of Zoon's balanitis.[6]
Histopathology
ZB has distinctive histopathological features, which include:[7]
Epidermal
- Epidermal changes include early thickening with acanthosis and parakeratosis of epidermis, which is followed by atrophy, erosion and spongiosis.
- Scattered neutrophils may be found in superficial erosions of the epidermis.
- Spongiosis accentuation may occur in the lower half of spinous zone.
- Subepidermal clefts, necrotic keratinocytes, and lozenge keratinocytes may be seen in the later stages of Zoon's balanitis.
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 Zoon's Balanitis.
- In the later stages, upper dermis may show fibrosis which correlates well with subepidermal clefts, epidermal atrophy, and plasma cell infiltrates.
Epidemiology and Demographics
There is no comprehensive studies studying the epidemiology and demographics of Zoon's disease in general population. A recent study has reported that out of 226 patients examined in a genitourinary medicine clinic over a period of 3 years, about 26(10%) of patients were diagnosed with Zoon's balanitis.[8]
Screening
There is no established screening guidelines for Zoon's balanitis.
Natural History, Complications, and Prognosis
Natural history
If left untreated, patients with Zoon's balanitits may develop pain, phimosis, and paraphimosis. Studies have reported that there could be an increased risk of transformation of these lesions into Squamous cell carcinoma.[9]
Complications
Complications of Zoon's balanitis include:
- Phimosis
- Paraphimosis
- Risk of transformation into malignancy(Squamous cell carcinoma)[9]
Prognosis
Prognosis is usually good with treatment.[10]
Diagnosis
History and symptoms
Patients with Zoon's balanitits could present with asymptomatic or symptomatic lesion with:[11]
- Itching (pruritus) of the genitalia
- Discomfort in urination(dysuria)
- Pain in the genital region
- Blood stain discharge from the lesion
- Difficult or painful sexual intercourse
Physical examination
Characteristic lesions seen in Zoon's balanitis are:[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 affecting the glans penis. Inner surface of prepuce and coronal sulcus may also be involved.
- Though uncommon, lesions of Zoon's balanitis may also involve other sites which include labia minora in females, oral mucosa, conjunctiva, urethra, cheeks, and epiglottis.[13]
Clinical criteria for diagnosing Zoon's balanitis include the following:[12]
- Shiny, erythematous patches on the glans, prepuce, or both
- Lesions present for > 3 months
- Absence of lesions which are suggestive of Lichen planus or 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 in Zoon's balanitis include the following:[14][15]
Reflectance confocal microscopy
A nucleated honeycomb pattern and vermicular vessels is a clue for benign inflammatory genital skin disease
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.
Biopsy
Epidermis
Epidermal thickening which is followed by epidermal atrophy, at times with erosions
Dermis
Plasma cell infiltrate with haemosiderin and extravasated red blood cells.
Treatment
Management of Zoon's balanitis include general measures, medical and surgical modalities:[16][17][18]
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 Balanitis in general.[17]
Medical Therapy
| Medical modalities for treating Zoon's balanitis | |
|---|---|
| Drugs | Drug dosage |
| Topical steroids | Saline compresses containing 1% hydrocortisone/0.02% betamethasone+/-17-valerate/0.05% betamethasone dipropionate |
| Oxytetracycline 3%, nystatin 100,00(units/g), and clobetasone butyrate 0.05% applied until complete resolution was observed | |
| Topical calcineurin | Tacrolimus ointment 0.1% twice daily |
| Topical Pimecrolimus | Pimecrolimus cream 1% twice daily |
| Topical Imiquimod | 5% imiquimod cream, 3 times a week for 4 months with multiple periods without treatment |
| 5% imiquimod cream, 3 times a week for 12 months without any interruption | |
Surgery
| Surgical modalities for treating Zoon's balanitis | |
|---|---|
| Procedure | Follow-up |
| 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(Photodynamic therapy) | Lesion healed completely after an average 2.75 PDT sessions, with no relapse observed in following 1 year of follow up |
Prevention
Primary Prevention
There is no established primary prevention methods for prevention of Zoon's balanitits.
Secondary prevention
There is no established secondary prevention methods in preventing Zoon's balanitis.
References
- ↑ 1.0 1.1 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.
- ↑ Pearce J, Fernando I (2015). "The value of a multi-specialty service, including genitourinary medicine, dermatology and urology input, in the management of male genital dermatoses". Int J STD AIDS. 26 (10): 716–22. doi:10.1177/0956462414552695. PMID 25294843.
- ↑ 9.0 9.1 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.
- ↑ 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.
- ↑ 17.0 17.1 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.