Sandbox:Balanitis xerotica obliterans

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:BXO, Penile lichen sclerosus

Overview

Balanitis xerotica obliterans (BXO) is a dermatological (skin) condition affecting the male genitalia. It was first described by Stuhmer in 1928, though earlier reports describe what may have been the same condition.[1] BXO commonly occurs on the foreskin and glans penis.[2] Atrophic white patches appear on the affected area,[3] and commonly, a whitish ring of indurated (hardened) tissue usually forms near the tip that may prevent retraction.[2]

Classification

There is no established classification system for BXO.

Pathophysiology

The exact etiology of BXO is unknown, but multiple factors are considered to play an important in the development of BXO.

Factors associated with pathogenesis of BXO
Uncircumcised Penis	Accumulation of secretions and epithelial debris between the foreskin and coronal sulcus leads to chronic irritation, sublincal trauma.
Autoimmune diseases	Patients with BXO, were found to have an other associated autoimmune conditions, which include: diabetes mellitus, vitiligo, alopecia aerata. Some studies have showned association between BXO and HLA DQ7 with DR11 and DR12.
Infections	Human papillomavirus (HPV)	Several studies have implicated human papillomavirus as a causative agent in pathogenesis of BXO. HPV 16, 18, 33 and 51 have been found to associated with BXO. Recent studies reported lack of clincal correlation of BXO and HPV, has they both have unrelated transcriptosome.
	Several studies have reported association of various infectious organisms with development of Balanitis xerotica obliterans, which include: Borrelia burgdoferi HCV Epstein-Barr virus
Genetics	Several studies have proposed genetic association and lichen sclerosis. In females, 12% of patients were found to have a family history of lichen sclerosis,. In males, there is no evidence familial predisposition.
Environmental factors		BXO is known to demonstrate koebner phenomenon. Trauma, old scars, skin grafts, sunburn and radiation were found to be associated with BXO. Some studies have proposed that post-micturation dribbling or microincontinence plays a central role in development of BXO.

Histopathology

Histopatholgy findings found in BXO include:

Early stage of BXO

• Moderately heavy lymphocytic infiltrate in found in basal epidermis and superficial dermis in early stages of the lesion.

Late stages of BXO

• Epidermis becomes atrophic with surface hyperkeratosis, thickened basement membrane
• Broad zone of subepidermal oedema with homogenization of collagen, which becomes more sclerotic over time.
• In few cases, epidermis is detached from dermis resulting in formation of haemorrhagic bullae.
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Causes

The etiology of BXO is uncertain. However, some possibilities have been suggested:

Causes of BXO
Uncircumcised Penis

Epidemiology and Demographics

The true prevalence of BXO is controversial and unclear. One study calculated a rate of 0.6% of boys affected by their 15th birthday. Another reported a rate of 0.07%. However, a review noted that "with a high degree of suspicion and histologic examination, the condition will prove to be much more frequent than one generally believes." Another suggested that "more cases would be diagnosed during infancy if all dried foreskin were examined systematically." Another remarked that the condition "may be misdiagnosed or ignored in the young boy." Yet another commented that "its true incidence is not appreciated because most cases are cured by circumcision, and unfortunately many surgeons still fail to send their circumcision specimens for histology." Another remarked that the "extent of asymptomatic disease in this series would suggest the true prevalence of LS in men might be much higher than published work suggests."

According to some authors, the disease most frequently affects middle-aged men. However, a large study reported that the age distribution was similar from 2 to 90 years of age, except for men in their twenties, who were at twice the risk. The same study found that black and Hispanic men had approximately twice the risk of white men. The authors suggested possible reasons for this, including access to health care, differences in neonatal circumcision rates, and climate differences.

Mallon et al. found that BXO was related to circumcision status. Adjusting for age, lack of circumcision was associated with an odds ratio of 53.55. The finding was statistically significant. However, BXO has also been noted to occur after late circumcision, especially when performed for phimosis.

Screening

There is no established screening guidelines for BXO.

Natural History, Complications, and Prognosis

Natural history

If left untreated, BXO involve the penile skin, scrotum, and entire urethra leading to the complications.

Complications

Complication of BXO include the following:

• Phimosis
• Painful erection
• Reduced urinary flow
• Urinary retention
• Risk of malignant transformation into Squamous cell carcinoma(Long-term prospective studies are needed to determine the real risk of malignant transformation)

Prognosis

Prognosis is good with treatment.

Diagnosis

History and symptoms

Patients with BXO could be asymptomatic or present with:

Whitening or reddening of the penile region

• Difficulty in retracting the foreskin
• Painful erection
• Reduced urinary flow
• Urinary retention
• Buring sensation ( paraesthesia)(rare)
• purple rash in gential region( purpura)(rare)
• Small red or purple clusters, often spidery in appearance, on penis(telangiectases)(rare)

• Itching (pruritus) of the genitalia.rare
• Discomfort in urination(dysuria)rare

Physical examination

Physical examination findings include:

• Whitening or reddening of the glans penis, foreskin (prepuce) and coronal sulcus
• Induration of the glans and foreskin
• Phimosis(rare)
• Purpura(rare)
• Telangiectases(rare)

Laboratory findings

Tzanck smear and cutaneous biopsy, along with a rapid protein reagin test, will provide a definitive diagnosis."

Tzanck smear
Cutaneous biopsy

Treatment

Medical therapy for BXO include:

Various medical managements for BXO
	Drug dosage	Effectiveness
Topical steroids	Betamethasone diproprionate 0.05% or or clobetasol proprionate 0.05% cream or ointment once or twice daily After 6–8 weeks, reduce the application of the topical steroid to every second day After 12–16 weeks to assess response to treatment(mometasone aceponate 0.1% cream can be substituted if there is a good response) No improvement by 6 months, then use of the potent topical steroid should be abandoned.	3 out of 6 patients responded
Topical calineurin inhibitors	Tacrolimus ointment 0.1% twice daily	Shouldn't be used as first-line therapy
	Pimecrolimus cream 1% twice daily
Tricyclic antidepressant or gabapentin.	Can be used in cases when BOX is associated with penile dysaesthesia.
			Surgery
Surgical treatment often involves circumcision. Trial of steroids is usually prescribed before subjecting patients for surgery. Phimosis is an indication for surgery. In patients with severe BXO may require an extensive surgery with disease control, function and cosmesis in carefully balanced. Some cases may require meatoplasty, extensive urethroplasty and reconstructions. Patients who undergo surgery should be follow up as the disease as tendency to recur.(BOX as high tendency to recur due to koebner phenomenon) Patients should be advice for regular testicular self-examination and should be advice to return if the lesion revur.
Prospective therapies
Intralesional corticosteroids, topical and intramuscular testosterone, intravenous procaine, topical oestrogen and retinoid creams, oral vitamin E, radiation therapy and CO2 laser are currently been studies for there role in treating BOX

<section></section>Prevention

There is no known means of preventing BXO. However, one study reports that the data "suggest that circumcision prevents or protects against common infective penile dermatoses."

Primary Prevention

Circumcision in males can help in reducing risk of having BXO.

Secondary prevention

There is no secondary prevention measures.

References

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