This 76-year-old woman noted vaginal bleeding, which was initially diagnosed as lichen sclerosis. She then developed intermittent painful lesions in oral mucosa followed by dysphagia. Initially she had trouble swallowing pills, but ater she developed dysphagia with most foods, choking sensation, and retrosternal pain. She lost significant weight over 2-3 years. A biopsy of the gingiva was consistent with lichen planus, and endoscopy revealed a cervical esophageal stricture severe enough to prevent passage of the endoscope. A complete medical and laboratory evaluation was otherwise normal. She was treated with oral prednisone and mycophenolate mofetil and tacrolimus topically. Prednisone was tapered slowly. Serial esophageal dilations with intralesional steroid injections into stricture was also performed. Lichen planus (LP) is an idiopathic disease of the skin and mucosa. Mucosal surfaces involved in lichen planus may include perineum, oral mucosa and pharynx. Histologically, LP is characterized by a dense, band-like lymphocytic infiltrate under an acanthotic epidermis, destruction of the basal layer and hypergranulosis. Histology of mucosal epithelium in LP is slightly different than cutaneous LP. It frequently shows parakeratoisis and often fails to show hypergranulosis. The epithelium may be atrophic rather than acanthotic.
Oral lichen planus is seen in approximately 1-4 percent of the population, with onset of disease occurring most frequently in the 5th and 6th decade. Approximately 25 percent of all patients with LP have only mucosal manifestations. The most common form of oral LP is the reticular pattern, characterized by Wickham’s striae, that is usually asymptomatic. Erosive oral LP, on the other hand, is less common, but may be extremely painful.
Esophageal lichen planus is often under reported and diagnosed. It should always be evaluated for in patients with oral LP. Esophageal LP occurs mainly in middle-aged women. The overwhelming clinical complaint is dysphagia, but reflux type symptoms or a history of retrosternal pain can often be elicited. All of the patients, thus far reported, with esophageal disease have had concomitant oral LP.
Although the histology and clinical exam often make the diagnosis of lichen planus, the differential diagnosis of esophageal strictures with ulceration should include esophagitis from pill ingestion, GERD, infectious agents, and medication induced LP-like lesions (from gold, thiazides, and anti-malarials).
Correct diagnosis and treatment of esophageal LP is of critical importance. Delay in diagnosis may lead to serial dilations of esophageal strictures that, without simultaneous medical treatment, may lead to koebnerization of lichen planus and worsening of the stricture. Patients can lose significant weight and become dehydrated secondary to stenosis. In addition, patients should be monitored for transformation of oral LP lesions into squamous cell carcinoma, as this has been reported in several studies.
Therapy for oral lichen planus with esophageal involvement includes systemic as well as possible topical therapies. Referral to a gastroenterologist for endoscopy and dilations, with or without steroid injections, is often necessary. At first diagnosis, systemic steroids are often needed to quell the inflammation. A variety of steroid sparing agents have been used successfully including mycophenolate mofetil, azathioprine, cyclosporine, and etretinate. Topical steroids and tacrolimus may ease the symptoms.
References:
Abraham SC, Ravich WJ, Anhalt GJ, et al. Esophageal lichen planus: Case report and review of the literature. American Journal of Surg Path. 2000; 24(12): 1678-1682.
Menges M, Hohloch K, et al. Lichen planus with esophageal involvement. Digestion. 2002; 65: 184-189.
Harewood GC, Murray JA, Cameron AJ. Esophageal lichen planus: the Mayo Clinic experience. Dis Esophagus. 1999; 12(4): 309-311.
Description
The esophageal biopsy showed a focal erosion with acute and chronic inflammation. Reactive epithelial changes with acantholysis and a cleft compatible with bulla formation was also seen.
