Dermatology

Zoon’s balanitis (balanitis circumscripta plasmacellularis)

Zoon’s balanitis (balanitis circumscripta plasmacellularis) ICD-9 607.1

Are You Confident of the Diagnosis?

Characteristic findings on physical examination

The lesions are round to oval, and have a moist velvety appearance.

Expected results of diagnostic studies

Biopsy is required to establish the diagnosis. A biopsy of Zoon’s balanitis demonstrates atrophy of the epithelium with mild intraepithelial spongiosis surrounding flattened diamond-shaped keratinocytes. The underlying dermis demonstrates a dense lymphoplasmacytic infiltrate.

Diagnosis confirmation

The major differential is erythroplasia of Queyrat (a velvety red form of Bowen’s disease), and a biopsy is required to establish the diagnosis. Zoon’s balanitis is almost exclusively a disease of uncircumcised males, so the presence of a similar lesion in a circumcised individual is much more suspicious for malignancy.

Who is at Risk for Developing this Disease?

Zoon’s balanitis is a disease of older uncircumcised men.

What is the Cause of the Disease?

The etiology is unknown.

Systemic Implications and Complications

Similar lesions may be seen in the vulva and oral mucosa, but there are no internal manifestations.

Treatment Options

Circumcision is usually the most effective form of therapy. Refractory disease can show some improvement with topical corticosteroids, topical calcineurin inhibitors, and laser ablation.

Optimal Therapeutic Approach for this Disease

Once a biopsy has established the diagnosis, a trial of a corticosteroid or topical calcineurin inhibitor may be reasonable, but in the author’s experience they usually fail and most patients are ultimately cured via circumcision. Erb:Yag laser ablation has been used with anecdotal success and would be a reasonable third line option.

Patient Management

The condition should clear completely. Persistent disease should prompt a repeat biopsy.

Unusual Clinical Scenarios to Consider in Patient Management

Obese circumcised patients may develop disease because of retraction of the penis under skin folds. Weight loss, topical therapy and laser ablation represent treatment options, but these patients are often refractory to treatment.

What is the Evidence?

Wollina, U. "Ablative erbium: YAG laser treatment of idiopathic chronic inflammatory non-cicatricial balanoposthitis (Zoon's disease) - a series of 20 patients with long-term outcome". J Cosmet Laser Ther. vol. 12. 2010 Jun. pp. 120-3.

(An uncontrolled trial was performed with 20 Caucasian male patients (mean age 64.8 years) who presented with chronic inflammatory non-cicatricial balanitis or balanoposthitis. In all patients a complete re-epithelialization could be achieved within 2-3 weeks. During follow-up between 3 months and 30 months (mean 12.1+/- 7.2 months), a complete and stable clearing was achieved in 20 patients (100%). There were no severe adverse effects.)

Brix, WK, Nassau, SR, Patterson, JW, Cousar, JB, Wick, MR. "Idiopathic lymphoplasma cellular mucositis-dermatitis". J Cutan Pathol. vol. 37. 2010 Apr. pp. 426-31.

(A uniform nomenclature for Zoon-like lesions does not exist. Based on the authors' analysis, they suggest that the generic term idiopathic lymphoplasmacellular mucositis-dermatitis be considered to encompass the lymphoplasmacellular infiltrates in the skin and mucosal surfaces considered herein. This designation is morphologically descriptive and can be applied regardless of anatomic location.)

Stinco, G, Piccirillo, F, Patrone, P. "Discordant results with pimecrolimus 1% cream in the treatment of plasma cell balanitis". Dermatology. vol. 218. 2009. pp. 155-8.

(The authors reported three cases of plasma cell balanitis refractory to several treatments with steroids and antifungals treated with pimecrolimus1% cream applied twice daily: one patient had a complete resolution, one patient had a marked response but relapsed during the treatment and the last patient had a partial response due to the development of a side effect that precociously required to stop the treatment.One patient referred a slight pruritus after the first applications of the cream that spontaneously disappeared after a few minutes. Additional experiences are needed to determine if topical pimecrolimus is an effective and safe treatment for plasma cell balanitis.)

Moreno-Arias, GA, Camps-Fresneda, A, Llaberia, C, Palou-Almerich, J. "Plasma cell balanitis treated with tacrolimus 0.1%". Br J Dermatol. vol. 153. 2005 Dec. pp. 1204-6.

(Two uncircumcised mature Caucasian males were seen, both presenting with Zoon's balanitis; topical tacrolimus 0.1% ointment twice daily was prescribed in each case. Marked improvement of the lesions in both patients has been observed, with follow-up of 1 year and 10 months, respectively.)

Santos-Juanes, J, Sánchez del Río, J, Galache, C, Soto, J. "Topical tacrolimus: an effective therapy for Zoon balanitis". Arch Dermatol. vol. 140. 2004 Dec. pp. 1538-9.

(The authors reported three cases of ZB that responded well to treatment with topical tacrolimus that was utilized for 3 weeks. One patient required 2 more weeks of treatment for erythema of the prepuce. The authors concluded that topical tacrolimus is an effective means of controlling the signs and symptoms of diverse inflammatory mucous membrane and genital diseases such as erosive or ulcerative oral lichen planus, oral cicatricial pemphigoid, and anogenital lichen sclerosus, with no notable adverse effects.)

Alessi, E, Coggi, A, Gianotti, R. "Review of 120 biopsies performed on the balanopreputial sac. From zoon's balanitis to the concept of a wider spectrum of inflammatory non-cicatricial balanoposthitis". Dermatology. vol. 208. 2004. pp. 120-4.

(120 balanopreputial sac biopsies performed at the surgical unit of the authors' institute from January 1999 to December 2002 were reviewed, examining also the patients' clinical records. The authors found that a small group of previously unclassified cases showed common clinical and histopathological features. Clinically, all patients were uncircumcised and had long-lasting asymptomatic erythematous plaques on the balanopreputial sac with no erythroplastic or lichenoid features and no correlation with sexual intercourse.Histologically, all specimens showed a thinned and spongiotic epithelium, a band-like infiltrate of lymphocytes and histiocytes with a variable number of plasma cells in the upper part of the chorion and further signs of acute, subacute or chronic inflammation. The authors believe that these cases fall within a spectrum of inflammatory non-cicatricial disorders, ranging from almost pure lymphohistiocytic forms to forms which fulfil all criteria to be classified as balanitis circumscripta plasmacellularis of Zoon. They propose to classify these cases histologically as inflammatory non-cicatricial balanoposthitis and clinically as idiopathic inflammatory non-cicatricial balanoposthitis.)
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