Sandbox: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

Overview

Zoon's balanitis is an idiopathic, chronic, benign inflammatory mucositis of the genitalia. 

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.
• In 1954, Garnier reported the similar lesion in vulva.
• In 1956, Nikolowski described the identical lesion in oral mucosa.
• In 1963, Kortnig described the idential lesion in conjuntiva.

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:

• 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.
• 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.

Histopathology

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.
• 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

Diagnosis

Clinical criteria in diagnosing Zoon balanitis
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.

History and symptoms

Patients with Zoon balanitits could be asymptomatic or present with:

• 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:

The lesions are 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)

To view an image

It presents as single or multiple well-circumscribed, orange-red, shiny, moist, glistening macular to slightly raised plaque(s) [Figures ​[Figures11 and ​and2].2]. The clinical criteria given by Kumar et al. are given in Table 1.[18] Multiple pinpoint, brighter red spots may be speckled on the background of this orange-red plaque, called as “cayenne pepper spots” due to microhemorrhage and hemosiderin deposition.[1] Sometimes, prepuce may display “kissing lesion” on areas that is in direct contact with the lesions. Subsequently, these may erode and leave behind a “rusty stain.”[1] Two distinct clinical variants of ZB have been reported, which include erosive type with bleeding, particularly on the inner surface of prepuce and vegetative type.[11]

Laboratory findings

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.