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Balanitis Circumscripta Plasmacellularis

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
George C Keough, MD, Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center; Daniel S Lehman, MD, Fellow in Minimally Invasive Urology/Oncology, Department of Urology, Columbia University Medical Center

Updated: Mar 17, 2008

Introduction

Background

In 1952, J.J. Zoon1 first recognized balanitis circumscripta plasmacellularis or plasma cell balanitis (PCB) is an idiopathic, rare, benign penile dermatosis. It is important to distinguish this benign condition from the clinically similar neoplastic erythroplasia of Queyrat.

Immunohistologically, immunoglobulin E and immunoglobulin G are found to be major immunoglobulin classes in the plasma cellular infiltrate. Immunoglobulin M–positive cells are either absent or present in very low numbers. Although a specific allergen has not been identified, the findings suggest that this condition could be related to immediate hypersensitivity.

The kappa-to-lambda ratio is variable. This suggests a nonspecific polyclonal stimulation of B cells in the basis of PCB, which might be caused by a persistent infection.

Other related eMedicine articles include Bowen Disease, Lichen Sclerosus et Atrophicus, Erythroplasia of Queyrat (Bowen Disease of the Glans Penis), Balanitis Xerotica Obliterans, and Balanitis. In addition, the Medscape CME course HPV-Related Disease in Men: Anal and Penile Cancer (Slides With Transcript) may be helpful.

Frequency

International

Mallon et al2 studied 357 male referral patients with genital skin disease. They studied a control group of 305 male dermatology clinic patients without genital skin disease over a 120-day period. Their goal was to investigate the relationship between circumcision and penile disease. Mallon et al2 compiled the percentage of circumcision in the general male dermatology population. Zoon balanitis occurred in 27 patients. More patients had squamous cell carcinoma, bowenoid papulosis, and Bowen disease. They noted that every patient with Zoon balanitis, bowenoid papulosis, and nonspecific balanoposthitis had not been circumcised.

Sex

PCB affects males. Analogous lesions sharing both clinical and histologic features of PCB have been reported in women as vulvitis circumscripta plasmacellularis.

Age

PCB is most common in middle-aged to older men, with cases reported in patients aged 20-88 years.

Clinical

History

The patient, a male of middle age or older, usually presents with a characteristic lesion of the glans penis or prepuce, present for an average of 1-2 years before diagnosis. Symptoms are minimal, but patients may complain of mild pruritus or tenderness. Some patients present for evaluation because of cosmetic concerns or anxiety. Bloodstaining of the underclothes for 5 months prior to presentation has been reported in a patient with PCB. In 2007, Toker et al3 reported on Zoon balanitis in a circumcised man.

Physical

The PCB lesion is usually a solitary, glistening, shiny, red-to-orange plaque of the glans or prepuce of an uncircumcised male. The lesions may exhibit a yellowish hue with pinpoint purpuric "cayenne pepper" spotting. Erosive and vegetative variants have been reported. Bowen disease of the glans penis (erythroplasia of Queyrat) has been reported in association with PCB; thus, attention must be given to possible neoplastic associations with this condition.

Kumar et al4 studied 112 persons with a clinical diagnosis of PCB ranging in age from 24-70 years. Most had been symptomatic for more than 12 months. Plaques manifested on the prepuce and glans in 58.92% of patients, in the prepuce only in 23.21% of patients, and on the glans only in 17.85% patients.

Causes

The etiology of PCB is unknown. Importantly, all confirmed cases have been in uncircumcised males. It has been proposed that friction, trauma, heat, poor hygiene, chronic infection with Mycobacterium smegmatis, a reactive response to an unknown exogenous or infectious agent, an immediate hypersensitivity response mediated by immunoglobulin E class antibodies, and hypospadias may be predisposing or inciting agents. There is no evidence of human papilloma virus infection in PCB.

Differential Diagnoses

Candidiasis, Mucosal
Pemphigus Vulgaris
Erythroplasia of Queyrat (Bowen Disease of the Glans Penis)
Psoriasis, Plaque
Herpes Simplex
Syphilis
Lichen Planus
Warts, Genital
Lichen Sclerosus et Atrophicus

Other Problems to Be Considered

Fixed drug eruption
Erosive lichen planus
Erosive balanitis
Solitary plasmocytoma
Malignant plasma cell tumors in HIV-infected patients
Syphilis: In 2007, Babu et al5 noted primary syphilis presenting as balanitis.

Workup

Laboratory Studies

  • Diagnosis of PCB is confirmed by distinctive histologic findings upon skin biopsy.

Procedures

  • Skin biopsy

Histologic Findings

Skin biopsy of the PCB lesion reveals suggestive, if not distinctive, changes in the epidermis and dermis. There is epidermal thinning with effacement of the rete ridges. This atrophy may be significant enough to reveal ulceration of the epidermis. Diamond-shaped or lozenge keratinocytes are common. There is also uniform spongiosis, known as "watery spongiosis." Dyskeratotic keratinocytes are not uncommon.

Dermal findings include a dense subepidermal infiltrate with a predominance of plasma cells. Vascular proliferation with erythrocyte extravasation and hemosiderin deposits is noted frequently.

Alessi et al6 analyzed this condition and found that a small group of previously unclassified cases showed common clinical and histopathological features. All of the patients were uncircumcised, and all had long-standing asymptomatic erythematous plaques on the balanopreputial sac. No correlation with sexual intercourse was reported. Histologically, all the specimens showed a thinned and spongiotic epithelium, a bandlike infiltrate of lymphocytes, and histiocytes. A variable number of plasma cells were present in the upper part of the submucosa. The authors concluded that these cases fell within a spectrum of inflammatory noncicatricial disorders, ranging from almost pure lymphohistiocytic forms to forms that fulfill all criteria for balanitis circumscripta plasmacellularis of Zoon. They proposed the term idiopathic inflammatory noncicatricial balanoposthitis.

Weyers et al7 studied 45 cases of balanitis of Zoon clinically and histopathologically. They noted that slight epidermal acanthosis and parakeratosis and a patchy lichenoid infiltrate of lymphocytes and some plasma cells were present early. They also reported epidermal atrophy and erosions, a scattering of neutrophils in the upper reaches of the epidermis, scant spongiosis, RBC extravasation, and a much denser infiltrate with many plasma cells as late findings in one case. Late changes included subepidermal clefts, ulceration, superficial dermal marked fibrosis, and a marked increase of siderophages.

These findings suggested that PCB results from irritation or mild trauma involving scantly keratinized skin in a moist environment. Weyers et al also noted that balanitis of Zoon may be found superimposed on the skin eruptions of other types of cutaneous pathology and that it can alter such histopathology. Some eruptions diagnosed as PCB included allergic contact dermatitis, psoriasis, lichen planus, and Bowen disease. Their conclusion was that PCB is simply a reaction pattern.

Of the 112 persons with a clinical diagnosis of PCB studied by Kumar et al,4 96 underwent histopathological tissue analysis. Histological features included (1) epidermal edema; (2) a dense upper dermal band of chronic inflammatory cells, including many plasma cells; (3) dilated capillaries and extravasated red blood cells; and (4) hemosiderin deposition.

Treatment

Medical Care

Topical tacrolimus ointment has been reported useful for Zoon balanitis.8,9,10 In 2008, Virgili et al11 reported in a comparative analysis of subjective, objective, and histopathological data that topical tacrolimus was less effective in treating plasma cell vulvitis compared with Zoon balanitis in men.

Also in 2008, Bardazzi et al12 evaluated pimecrolimus 1% cream for treating resistant Zoon balanitis in 2 patients. One patient had a complete regression of the lesion after 2 months of therapy, and the other had great improvement of the lesion but a hyperpigmented patch persisted on the glans.

In 2007, Nasca et al13 described a 43-year-old, uncircumcised, white, diabetic man with a 4-year history of Zoon balanitis that was unresponsive to topical steroid therapy. He experienced a clinical but not histological cure after 16 weeks of thrice-weekly imiquimod 5% therapy, with no relapses at 18-month follow-up. A moderate-to-marked cutaneous inflammation occurred several times during treatment, which required rest periods of several days' duration.

Fusidic acid cream 2% has been reported as effective in disease suppression and curative in some patients.14 Topical agents such as corticosteroids, antibacterials, gentian violet, and antifungal agents have been used with only limited success in patients with PCB and are not curative. Intralesional interferon alpha was found to be helpful in the treatment of the vulvar analog of PCB.15 Griseofulvin therapy and oral tetracycline have been tried without success.

Surgical Care

The treatment of choice for PCB is circumcision, which is curative.16,17,18 Patients must be informed that circumcision is the current criterion standard for the treatment of this disorder. The carbon dioxide laser has been used successfully in ablation of PCB lesions.19 Retamar et al20 treated 5 patients with a carbon dioxide laser, and 3 were free from disease years later. Radiotherapy and electrodesiccation have been used with less than optimal results.

Palminteri et al21 noted that in selected cases of benign, premalignant, or malignant penile lesions (including that related to Zoon balanitis), glans resurfacing or reconstruction can ensure a normal-appearing and functional penis, without jeopardizing cancer control.

Consultations

Consultation with a urologist may be helpful.

Medication

Topical calcineurin inhibitors

Topical tacrolimus inhibits calcineurin, which is related to stimulation by IL-2 of T-cells. As such, it is an anti-inflammatory agent.


Tacrolimus ointment 0.1% or 0.03% (Protopic)

Topical tacrolimus ointment inhibits calcineurin, which is related to stimulation by IL-2 of T-cells. As such, it is an anti-inflammatory agent.

Dosing

Adult

Apply qd/bid

Pediatric

Tacrolimus ointment 0.03% is approved for treatment of moderate-to-severe atopic dermatitis

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

FDA black box warning states tacrolimus may increase risk of developing skin cancer; systemic absorption has been reported in patients with Netherton syndrome using topical tacrolimus

Follow-up

Prognosis

  • The prognosis is excellent if appropriate treatment is rendered.

Patient Education

  • For excellent patient education resources, visit eMedicine's Men's Health Center. Also, see eMedicine's patient education article Foreskin Problems.

Miscellaneous

Medicolegal Pitfalls

  • Failure to biopsy this lesion may lead to inappropriate medical or surgical therapy. This benign condition must be differentiated from penile carcinoma and erythroplasia of Queyrat to avoid overly aggressive therapy.

References

  1. Zoon JJ. [Chronic benign circumscript plasmocytic balanoposthitis.]. Dermatologica. 1952;105(1):1-7. [Medline].

  2. Mallon E, Hawkins D, Dinneen M, Francics N, Fearfield L, Newson R, et al. Circumcision and genital dermatoses. Arch Dermatol. Mar 2000;136(3):350-4. [Medline].

  3. Toker SC, Baskan EB, Tunali S, Yilmaz M, Karadogan SK. Zoon's balanitis in a circumcised man. J Am Acad Dermatol. Aug 2007;57(2 Suppl):S6-7. [Medline].

  4. Kumar B, Narang T, Dass Radotra B, Gupta S. Plasma cell balanitis: clinicopathologic study of 112 cases and treatment modalities. J Cutan Med Surg. Jan-Feb 2006;10(1):11-5. [Medline].

  5. Babu CS, Vitharana S, Higgins SP. Primary syphilis presenting as balanitis. Int J STD AIDS. Jul 2007;18(7):497-8. [Medline].

  6. Alessi E, Coggi A, Gianotti R. Review of 120 biopsies performed on the balanopreputial sac. from zoon's balanitisto the concept of a wider spectrum of inflammatory non-cicatricial balanoposthitis. Dermatology. 2004;208(2):120-4. [Medline].

  7. Weyers W, Ende Y, Schalla W, Diaz-Cascajo C. Balanitis of Zoon: a clinicopathologic study of 45 cases. Am J Dermatopathol. Dec 2002;24(6):459-67. [Medline].

  8. Hernandez-Machin B, Hernando LB, Marrero OB, Hernandez B. Plasma cell balanitis of Zoon treated successfully with topical tacrolimus. Clin Exp Dermatol. Sep 2005;30(5):588-9.

  9. Roé E, Dalmau J, Peramiquel L, Pérez M, López-Lozano HE, Alomar A. Plasma cell balanitis of zoon treated with topical tacrolimus 0.1%: report of three cases. J Eur Acad Dermatol Venereol. Feb 2007;21(2):284-5. [Medline].

  10. Moreno-Arias GA, Camps-Fresneda A, Llaberia C, Palou-Almerich J. Plasma cell balanitis treated with tacrolimus 0.1%. Br J Dermatol. Dec 2005;153(6):1204-6. [Medline].

  11. Virgili A, Mantovani L, Lauriola MM, Marzola A, Corazza M. Tacrolimus 0.1% Ointment: Is It Really Effective in Plasma Cell Vulvitis?. Report of Four Cases. Dermatology. Jan 9 2008;216(3):243-6. [Medline].

  12. Bardazzi F, Antonucci A, Savoia F, Balestri R. Two cases of Zoon's balanitis treated with pimecrolimus 1% cream. Int J Dermatol. Feb 2008;47(2):198-201. [Medline].

  13. Nasca MR, De Pasquale R, Micali G. Treatment of Zoon's balanitis with imiquimod 5% cream. J Drugs Dermatol. May 2007;6(5):532-4. [Medline].

  14. Petersen CS, Thomsen K. Fusidic acid cream in the treatment of plasma cell balanitis. J Am Acad Dermatol. Oct 1992;27(4):633-4. [Medline].

  15. Morioka S, Nakajima S, Yaguchi H, Naito K, Iwahara K, Ogawa H. Vulvitis circumscripta plasmacellularis treated successfully with interferon alpha. J Am Acad Dermatol. Nov 1988;19(5 Pt 2):947-50. [Medline].

  16. Ferrandiz C, Ribera M. Zoon's balanitis treated by circumcision. J Dermatol Surg Oncol. Aug 1984;10(8):622-5. [Medline].

  17. Kumar B, Sharma R, Rajagopalan M, Radotra BD. Plasma cell balanitis: clinical and histopathological features-- response to circumcision. Genitourin Med. Feb 1995;71(1):32-4. [Medline].

  18. Sonnex TS, Dawber RP, Ryan TJ, Ralfs IG. Zoon's (plasma-cell) balanitis: treatment by circumcision. Br J Dermatol. May 1982;106(5):585-8. [Medline].

  19. Baldwin HE, Geronemus RG. The treatment of Zoon's balanitis with the carbon dioxide laser. J Dermatol Surg Oncol. May 1989;15(5):491-4. [Medline].

  20. Retamar RA, Kien MC, Chouela EN. Zoon's balanitis: presentation of 15 patients, five treated with a carbon dioxidelaser. Int J Dermatol. Apr 2003;42(4):305-7. [Medline].

  21. Palminteri E, Berdondini E, Lazzeri M, Mirri F, Barbagli G. Resurfacing and reconstruction of the glans penis. Eur Urol. Sep 2007;52(3):893-8. [Medline].

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  25. Erdogan BS, Demirkan N, Aktan S, Ergin S, Evliyaoglu D. A focus on differential diagnosis of lichen planus and plasma cell balanitis. J Eur Acad Dermatol Venereol. Jul 2006;20(6):746-8. [Medline].

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  37. White JW Jr, Olsen KD, Banks PM. Plasma cell orificial mucositis. Report of a case and review of the literature. Arch Dermatol. Nov 1986;122(11):1321-4. [Medline].

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Keywords

plasma cell balanitis, Zoon balanitis,  plasma cell mucositis

Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

George C Keough, MD, Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center
George C Keough, MD is a member of the following medical societies: American Academy of Dermatology and American Medical Association
Disclosure: Nothing to disclose.

Daniel S Lehman, MD, Fellow in Minimally Invasive Urology/Oncology, Department of Urology, Columbia University Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Janet Fairley, MD, Professor, Program Director, Section Chief, Department of Dermatology, Medical College of Wisconsin
Janet Fairley, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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