Background
In 1952, JJ Zoon first recognized balanitis circumscripta plasmacellularis as a chronic, idiopathic, rare, benign penile dermatosis associated with dysfunctional foreskin in uncircumcised males. [1] Balanitis circumscripta plasmacellularis is also known as Zoon balanitis and plasma cell balanitis. The differential can be broad and includes a variety of sexually transmitted infections, drug eruption, psoriasis, and others. [2] It is important to distinguish balanitis circumscripta plasmacellularis, a benign condition, from the neoplastic process erythroplasia of Queyrat, an additional differential diagnosis that often leads to the biopsy of plasma cell balanitis. While plasma cell balanitis is considered a benign entity, a small number of case reports suggest that it may be a premalignant condition, but further study is required. [3]
Immunohistologically, IgE and IgG are found to be major immunoglobulin classes in the plasma cellular infiltrate. IgM- or IgA-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. [4] The kappa-to-lambda ratio is variable. This suggests a nonspecific polyclonal stimulation of B cells as the basis of balanitis circumscripta plasmacellularis (plasma cell balanitis), which might be caused by a persistent infection, although this theory has yet to be proven in the literature.
While the etiology remains unclear, it is believed that plasma cell balanitis is caused by dysfunctional foreskin, as it typically does not occur in circumcised men, with few exceptions. The dysfunctional foreskin is thought to cause retention of urine and smegma, trap heat and moisture, and predispose to chronic infection leading to irritation. [2]
Other related Medscape Drugs & Diseases articles include Bowen Disease, Lichen Sclerosus et Atrophicus, Erythroplasia of Queyrat (Bowen Disease of the Glans Penis), Balanitis Xerotica Obliterans, and Balanitis in Emergency Medicine.
Etiology
The etiology of balanitis circumscripta plasmacellularis (plasma cell balanitis) is unknown. It is important to note that 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 IgE class antibodies, and hypospadias may be predisposing or inciting agents. No evidence suggests human papillomavirus infection in plasma cell balanitis. [5] A 2019 case-control study showed an association with smoking and poor hygiene in patients with plasma cell balanitis. [6] Numerous authors also attribute poor hygiene to the development of plasma cell balanitis.
Epidemiology
Frequency
Balanitis circumscripta plasmacellularis (plasma cell balanitis) is an uncommon entity, but it is likely underreported owing to the lack of symptoms and hesitancy to perform a biopsy on the involved region. Mallon et al studied 357 male referral patients with genital skin disease over a 120-day period. [7] Plasma cell balanitis occurred in 27 patients (~7.6%). More patients had squamous cell carcinoma, bowenoid papulosis, and Bowen disease. They noted that every patient with plasma cell balanitis, bowenoid papulosis, and nonspecific balanoposthitis had not been circumcised. Pearce and Fernando evaluated 226 patients over a 3-year period. [8] Plasma cell balanitis occurred in 26 patients (~10%). A case of plasma cell balanitis has been noted in Cotonous (Benin) in an HIV-positive man who was circumcised. [9]
Sex
Balanitis circumscripta plasmacellularis (plasma cell balanitis) affects males. Analogous lesions sharing both clinical and histologic features of balanitis circumscripta plasmacellularis (plasma cell balanitis) have been reported in women as vulvitis circumscripta plasmacellularis.
Age
Balanitis circumscripta plasmacellularis (plasma cell balanitis) is most common in men of middle age or older, with cases reported in patients aged 20-88 years. In a 2018 report, three infants were diagnosed with clinical and dermoscopic findings consistent with plasma cell balanitis and responded to treatment consistent with the diagnosis. [10]
Prognosis
The prognosis is excellent if appropriate treatment is rendered.
Patient Education
For patient education resources, visit the Men's Health Center. Also, see the patient education article Foreskin Problems.
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Plasmacytosis mucosae of balanitis circumscripta plasmacellularis (plasma cell balanitis or Zoon balanitis. Hematoxylin and eosin stain. Courtesy of Nephron, via Wikimedia Commons.
