CASE REPORT Lichen Planus Involving the Esophagus ANDRZEJ UKLEJA, MD, KENNETH R. DeVAULT, MD, FACG, MARK E. STARK, MD, and SAMI R. ACHEM, MD, FACP, FACG KEY WORDS: esophagus; lichen planus; esophageal stricture; dysphagia. Lichen planus (LP) is a unique inflammatory disease of the skin, mucous membranes, nails and hair of unknown pathogenesis.[1, 2] The disorder can involve the conjunctivae, pharynx, esophagus, stomach, rec- tum, anus and bladder. The classic cutaneous lesions consist of violaceous, flat-topped papules with predi- lection for flexural areas of wrists, arms and legs. A unique feature of LP is the appearance of the lesions at sites of injured or traumatized skin referred to as the Koebner phenomenon. Frequently, LP has a chronic course with high relapse rate. The incidence of LP is between 0.1 to 1.2% of all skin diseases. Involvement of the esophagus in LP is rare, and only limited data are available in the literature. The pur- pose of this article is to report two cases of esopha- geal LP, both representing diagnostic challenges, and to review the literature on this subject including all published cases to date. CASE REPORTS Case 1. A 66-year-old white woman was referred for evaluation of recurrent esophageal dysphagia to solids for four years. She denied weight loss, reflux-type symptoms, and odynophagia. Her gastroenterologist treated her with sucralfate, omeprazole, and cisapride, and performed esophageal dilatations, which helped her symptoms tempo- rarily. The patient was referred to our institution for con- sideration of laparoscopic antireflux surgery. A 24-h pH study off acid suppressive drugs showed normal acid expo- sure. Esophageal motility was normal except for a hyper- tensive lower esophageal sphincter. Upper endoscopy re- vealed desquamating esophagitis involving the entire length of the esophagus. Sheets of squamous mucosa were peeling off from the esophageal wall, leaving the underlying mucosa friable and erythematous (Figure 1). No obvious stricture was noted, although the entire esophagus had a subtle narrowing. The stomach and duodenum were normal. Mul- tiple biopsies of the esophagus revealed severe acute/ chronic inflammation with squamous mucosa separation from inflamed submucosa (Figure 2). Cultures and stains for viral agents and fungi were negative. Upon further questioning, the patient admitted to a history of recurrent gingival lesions for many years. De- tailed examination of the oral cavity revealed mildly ery- thematous lesions in the periodontal areas. The examina- tion of larynx revealed marked erythema. The patient had suffered from dyspareunia and recurrent vaginitis for two years. She was treated unsuccessfully with various vaginal creams. A narrowed vagina with fusion of anterior and posterior walls was found on pelvic exam. A raised erythem- atous patch on the skin of the trunk was biopsied confirm- ing the diagnosis of LP. Repeated upper endoscopy two months later revealed multiple ring-type esophageal strictures starting at 20 cm from incisors, which were dilated with balloon and Savory dilators. Multiple ulcerations and diffuse desquamative changes were encountered once again, involving the entire length of the esophagus. Treatment with oral steroids (prednisone 30 mg/day) was initiated. At the six-month follow-up, the patient noticed significant improvement in her dysphagia, and a steroid tapering program was implemented. Case 2. A 65-year-old white female presented with dys- phagia and odynophagia without history of reflux for one year. Endoscopy showed severe esophagitis with a peculiar abnormality characterized by streaks of erythema extending 25–35 cm from the incisors without obvious stricture. No improvement was seen with omeprazole 20 mg daily. Re- peat endoscopy six months later showed friable mucosa peeling away from the underlying esophageal wall through- out the entire esophagus. The pathology was consistent with severe chronic esophagitis with a marked increase in reac- tive intraepithelial lymphocytes. The patient had no history or evidence of mucocutaneus lichen planus at the time of presentation. A year later, she developed vaginal burning and discharge not responding to antifungal and steroid creams, which finally led to the biopsy of the vagina and the diagnosis of LP. Repeated biopsies of the esophagus were consistent with lichen planus. Treatment with oral pred- nisone 30 mg/day was initiated; however, no improvement was seen over the five-month period. Dysphagia to solids became more prominent and multiple esophageal dilata- tions were performed. The patient initially lost 50 lb over a three-year period due to esophageal symptoms. She contin- ues to suffer from recurrent dysphagia and requires one to Manuscript received October 26, 2000; accepted February 29, 2001. From the Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Florida. Address for reprint requests: Dr. Sami R. Achem, Mayo Clinic, 4500 San Pablo Road, Jacksonville, Florida 32224. Digestive Diseases and Sciences, Vol. 46, No. 10 (October 2001), pp. 2292–2297 (© 2001) 2292 Digestive Diseases and Sciences, Vol. 46, No. 10 (October 2001) 0163-2116/01/1000-2292$19.50/0 © 2001 Plenum Publishing Corporation