Squamous cell carcinoma arising in long-standing lichen sclerosus et atrophicus.

Abstract

To the Editor: Lichen sclerosus et atrophicus is a chronic inflammatory lesion of the skin of unknown etiology. The condition is located most commonly in the genital regions, but extragenital sites may also occur. Hallopeau described the first report of lichen sclerosus in 1887.1 Clinically, the lesions begin as small white papules, progressing to large areas of pearly white, smooth, glistening plaques.2 It begins asymptomatically in most patients, with subsequent development of itching, pain, texture changes, and scarring. The histological changes of lichen sclerosus include edema, chronic inflammatory infiltrates, and dense homogenization of the dermis. The epidermis is atrophic, with hyperkeratosis and follicular plugging. Lichen sclerosus can be grouped with other immunologically mediated skin diseases including lichen planus, graft-versus-host disease, and lupus erythematosus.3 It can affect men and women and is found in all age groups. There is no general agreement concerning the exact relationship between anogenital lichen sclerosus and squamous cell carcinoma. Multiple studies have shown a 5% to 61% risk of lichen sclerosus developing into squamous cell carcinoma in patients with long-standing disease.4–6 We describe a case of vulvar squamous cell carcinoma developing in an area of preexisting lichen sclerosus to emphasize closer clinical follow-up of older patients diagnosed with this inflammatory disorder. An 81-year-old white female with a history of hypertension, type 2 diabetes mellitus, and vulvar lichen sclerosus et atrophicus presented to her dermatologist with a 6-month history of pruritus and irritation in the perineal region. The symptoms were becoming progressively uncomfortable. The lesion had been unresponsive to various topical glucocorticoid medications. When she had been diagnosed with lichen sclerosus 30 years ago, the lesion was treated with chloroquine, and complete resolution was noted. There was no recurrence of the lesion at a 5-year follow-up by her dermatologist. The patient noticed gradual increase in the severity of pruritus, hemorrhage, and discomfort. She had no other constitutional symptoms. Physical examination revealed hemorrhagic areas on both sides of the vulva and narrowing of the introitus. There was a large, ulcerated mass, 4 cm in diameter, in the area of the perineal body on the posterior aspect of the left labia. (Figure 1) No regional lymphadenopathy was identified. The remainder of the physical examination, routine laboratory studies, and chest radiograph was unremarkable. A vulvar shave biopsy from the center of the ulcerated mass was obtained and sent for microscopic evaluation. A diagnosis of invasive, well-differentiated squamous cell carcinoma was made. The patient was subsequently treated with palliative antipruritic medications. No excisional surgery or radiation therapy was performed. The patient continued to do well clinically during a follow-up of 9 months. Ulcerated, fissured, and hemorrhagic 4-cm mass of the perineal body. Note narrowing of the introitus. There is no general agreement concerning the exact relationship between anogenital lichen sclerosus and squamous cell carcinoma. Malignant changes are an accepted complication of certain inflammatory and scarring dermatoses.7 On the basis of a literature search (Medline 1966–2000), several studies were found of large cohorts of female patients with lichen sclerosus with a 4% to 5% risk of developing into squamous cell carcinoma in vulvar regions.4,5 Studies have shown the presence of adjacent lichen sclerosus in 25% of cases of vulvar squamous cell carcinoma.6 A follow-up study published confirmed similar results in 61% of patients in the study.8 The pathogenesis of squamous cell carcinoma arising from lichen sclerosus has not been fully elucidated. Changes in the local environment of the keratinocyte, including chronic inflammation and sclerosis, may be responsible for the promotion of carcinogenesis.9 The recommended management of lichen sclerosus includes potent topical corticosteroid ointment twice daily for 2 to 3 months.1 The dose should be gradually lowered and discontinued and used only to treat symptoms. The physician should check for nonhealing erosions, ulcerations, or warty lesions that may indicate development of malignancy. Long-term close follow-up is highly recommended. No topical androgens or systemic treatments are universally recommended. Surgery is not part of routine treatment, but it may be subsequently needed to treat symptoms of scarring or malignant changes. In conclusion, it has been shown that the risk of squamous cell carcinoma arising in a preexisting lichen sclerosis is high enough to mandate careful long-term follow-up of patients with this condition. It is also recommended that prompt biopsy of any clinically suspicious lesion be done and that patients who may not manifest any overt symptoms be treated. Early surveillance, detection, and treatment of squamous cell carcinoma arising in a preexisting lichen sclerosus et atrophicus will result in a decrease in morbidity and mortality rates for these older patients.