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Balanitis Circumscripta Plasmacellularis
Article Last Updated: Mar 17, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 10
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 is a member of the following medical societies: American Academy of Dermatology
Coauthor(s):
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
Editors: Janet Fairley, MD, Professor, Program Director, Section Chief, Department of Dermatology, Medical College of Wisconsin; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; 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; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
plasma cell balanitis, Zoon balanitis, plasma cell mucositis
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.
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.
Candidiasis, Mucosal
Erythroplasia of Queyrat (Bowen Disease of the Glans Penis)
Herpes Simplex
Lichen Planus
Lichen Sclerosus et Atrophicus
Pemphigus Vulgaris
Psoriasis, Plaque
Syphilis
Warts, Genital
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.
Lab Studies
- Diagnosis of PCB is confirmed by distinctive histologic findings upon skin biopsy.
Procedures
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.
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.
Drug Category: 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.
| Drug Name | Tacrolimus ointment 0.1% or 0.03% (Protopic) |
| Description | Topical tacrolimus ointment inhibits calcineurin, which is related to stimulation by IL-2 of T-cells. As such, it is an anti-inflammatory agent. |
| Adult Dose | Apply qd/bid |
| Pediatric Dose | Tacrolimus ointment 0.03% is approved for treatment of moderate-to-severe atopic dermatitis |
| Contraindications | Documented hypersensitivity |
| Interactions | None reported |
| 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 |
Prognosis
- The prognosis is excellent if appropriate treatment is rendered.
Patient Education
Medical/Legal 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.
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Balanitis Circumscripta Plasmacellularis excerpt Article Last Updated: Mar 17, 2008
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