Are You Confident of the Diagnosis?
The primary differential diagnosis is lichen planus, although Candida balanitis, psoriasis, and erythroplasia of Queyrat can also present with genital lesions that could be confused with balanitis xerotica obliterans (BXO). Itch is generally a prominent symptom. The absence of itch should prompt the clinician to question the diagnosis.
Characteristic findings on physical examination
On physical examination, lesions usually demonstrate atrophy and contraction of tissue. Loss of pigment is typically a prominent finding with a resulting white hue (Figure 1). The atrophic wrinkling characteristic of lichen sclerosus on other body sites is usually lacking in genital lesions.
Expected results of diagnostic studies
A biopsy is typically key to establishing the diagnosis. As with other forms of lichen sclerosus, BXO demonstrates hyperkeratosis, epidermal atrophy with loss of the rete pattern and a mild vacuolar interface dermatitis. The underlying dermis is homogenized and edematous in the upper portion, with an underlying sparse band of lymphoid cells. Pigment incontinence is usually present.
The key differential diagnosis is lichen planus, which may present with reticulated white lesions on an erythematous background, erosions, ulcerations or polygonal papules. Biopsy is key to an accurate diagnosis.
Who is at Risk for Developing this Disease?
Males and females of any age may develop lichen sclerosus. The term BXO is typically applied to adult men with atrophic disease. In children and adults, lichen sclerosus is a common cause of phimosis.
What is the Cause of the Disease?
Although the pathogenesis is unknown, HLA-subtype susceptibility and high rates of associated autoimmune disorders suggest that the disorder may be autoimmune in nature. Antibodies to extracellular matrix protein 1 (ECM1) have been identified, suggesting this protein may be the autoantigen involved.
Systemic Implications and Complications
This is a cutaneous disease and typically has no systemic manifestations. Chronic renal insufficiency has been reported in association with BXO, but a causal relationship remains to be established.
Potent topical corticosteroids are the mainstay of treatment. In general, the risk of atrophy from uncontrolled disease outweighs the risk of pulse therapy with potent topical corticosteroids. Potent topical corticosteroids, such as clobetasol, are typically applied daily for the first 2 weeks, then on weekends only. Topical calcineurin inhibitors, imiquimod and photodynamic therapy (PDT), and laser ablation (carbon dioxide laser, eximer and pulse dye lasers) have also been reported anecdotally to be effective. Photodynamic therapy can also be considered in treatment resistant cases.
Although topical testosterone was widely used in the past, controlled studies have shown it to be of no value. Circumcision may be appropriate for patients with phimosis, but many can respond to topical corticosteroids together with gentle stretching of the tissue.
Optimal Therapeutic Approach for this Disease
Begin treatment with clobetasol ointment applied daily at bedtime for 2 weeks. After the first 2 weeks, apply only on Saturday and Sunday each week. Refractory disease can be treated with longer cycles of corticosteroid use, but this would require close monitoring for atrophy secondary to the medication. Topical calcineurin inhibitors are reasonable as second line therapy, although some concern has been voiced about the potential for genital malignancy with these agents.
Patients should be monitored for symptoms, functional disability and atrophy. Atrophy is usually a sign of undertreated disease but could result from long-term daily application of topical corticosteroids. Pulse applications of corticosteroids (weekly) are preferred.
Unusual Clinical Scenarios to Consider in Patient Management
Undertreated patients are at risk for loss of tissue, loss of sexual function, and squamous cell carcinoma.
What is the Evidence?
McPherson , T, Cooper, S. “Vulval lichen sclerosus and lichen planus”. Dermatol Ther. vol. 23. 2010. pp. 523-32. (A good review of diagnosis and treatment options.)
Aidé , S, Lattario , FR, Almeida , G, do Val , IC, da Costa Carvalho, M. “Epstein-Barr virus and human papillomavirus infection in vulvar lichen sclerosus”. J Low Genit Tract Dis. vol. 14. 2010. pp. 319-22. (Although there is little evidence that human papillomavirus (HPV) plays a role, Epstein-Barr virus (EBV) was frequently found and deserves further study.)
Crispen , PL, Mydlo, JH. “Penile intraepithelial neoplasia and other premalignant lesions of the penis”. Urol Clin North Am. vol. 37. 2010. pp. 335-42. (Lichen sclerosus has a low risk of progression to malignancy.)
Olejek , A, Steplewska , K, Gabriel , A, Kozak-Darmas , I, Jarek , A, Kellas-Sleczka , S. “Efficacy of photodynamic therapy in vulvar lichen sclerosus treatment based on mmunohistochemical analysis of CD34, CD44, myelin basic protein, and Ki67 antibodies”. Int J Gynecol Cancer. vol. 20. 2010. pp. 879-87. (PDT may be considered as an option for some patients with lichen sclerosus.)
van Kessel , MA, van Lingen , RG, Bovenschen, HJ. “Vulvitis plasmacellularis circumscripta in pre-existing lichen sclerosus: treatment with imiquimod 5% cream”. J Am Acad Dermatol. vol. 63. 2010. pp. e11-3. (Rarely vulvitis plasmacellularis circumscripta [VCP] seen within lesions of lichen sclerosus. In this case the VCP responded to treatment with imiquimod.)
Bradford , J, Fischer, G. “Long-term management of vulval lichen sclerosus in adult women”. Aust N Z J Obstet Gynaecol. vol. 50. 2010. pp. 148-52. (Imiquimod represents a potential treatment option.)
Kiss , A, Király , L, Kutasy , B, Merksz, M. “High incidence of balanitis xerotica obliterans in boys with phimosis: prospective 10-year study”. Pediatr Dermatol. vol. 22. 2005. pp. 305-8. (Phimosis is a common sequela of lichen sclerosus in boys.)
Aynaud , O, Plantier, F. “Genital lichen sclerosus treated by carbon dioxide laser”. Eur J Dermatol. vol. 20. 2010. pp. 387-8. (Laser ablation represents a potential option for treatment.)
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