Zoon's balanitis

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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: ZB, Plasma cell balanitis

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,and named it has balanoposthite chronique circonscrite bénigne á plasmocytes or balanitis chronica circumscripta plasmacellularis. The exact pathogenesis of Zoon's balanitis is not clearly known. Patients with Zoon's balanitis presents with well circumscribed single or multiple orange-red in colour lesions with 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.
• In 1954, Garnier reported similar lesions in vulva.
• In 1956, Nikolowski described identical lesions in oral mucosa.
• In 1963, Kortnig described idential lesions in conjunctiva.

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:

• 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.

Histopathology

Zoon's balanitis has distinctive histopathological features, which include:

Epidermal

• Epidermal changes include early thickening with acanthosis and parakeratosis of epidermis, which is followed by atrophy, erosion and spongiosis of epidermis.
• 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 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 sub-epidermal clefts, epidermal atrophy, and plasma cell infiltrates.

Epidemiology and Demographics

There are 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.

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 balanitis 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.

Complications

Complications of Zoon's balanitis include:

• Phimosis
• Paraphimosis
• Risk of transformation into malignancy(Squamous cell carcinoma)

Prognosis

Prognosis is usually good with treatment.

Diagnosis

History and symptoms

Patients with Zoon's balanitis could present with asymptomatic or symptomatic lesions with:

• Itching (pruritis) in the genitalia region
• Discomfort in urination(dysuria)
• Pain in the genital region
• Blood stain discharge from the lesions
• Difficult or painful sexual intercourse

Physical examination

Characteristic lesions seen in Zoon's balanitis are:

• Well circumscribed single or multiple, orange-red in colour lesions with 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.

Clinical criteria for diagnosing Zoon's balanitis include the following:

• Shiny, erythematous patches on the glans, prepuceor both
• Lesions present for more than 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:

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 with fewer areas representing hemosiderin deposition and curved vessels due to epidermal thinning help 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 includes general measures, medical and surgical modalities:

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.

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 laser	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 measures for preventing of Zoon's balanitis.

Secondary prevention

There is no established secondary prevention measures for Zoon's balanitis.