Cyclosporine in the management of esophageal lichen planus

1Department of Medicine; 2Division of Pathology; 3Division of Dermatology; 4Division of Gastroenterology, Memorial University of Newfoundland, St John’s, Newfoundland Correspondence: Dr Mohsina Chaklader, University of Alberta, 1-10 Zeidler Ledcor Centre, 130 University Campus, Edmonton, Alberta T6G 2X8. Telephone 780-906-9775, fax 780-492-1600, e-mail mchak023@alumni.uottawa.ca Received for publication July 24, 2008. Accepted July 28, 2008 L planus (LP) is an uncommon disorder of unknown etiology mostly affecting women in their fifth and sixth decade of life. It is believed to be an autoimmune process involving T cells directed against basal keratinocytes, similar to graft-versus-host disease. It affects the skin, nails, oral pharynx, vulva and penis. Esophageal LP was first described in 1982 by Al-Shihabi and Jackson (1). LP involving the esophagus, stomach and rectum are rare. Esophageal LP can cause strictures, ulcerations and squamous cell cancer. The present article describes a case of LP, including history, physical examination and endoscopic findings, as well as the different treatment options available.

Lichen planus (LP) is an uncommon disorder of unknown etiology, mostly affecting patients in their fifth and sixth decade of life.It is believed to be an autoimmune process involving T cells directed against basal keratinocytes.It affects the skin, nails, oral pharynx and genitals.Esophageal involvement is quite rare and can cause strictures, ulcerations and squamous cell cancer.The present article describes the case of a 54-year-old woman who was referred for assessment of dysphagia that initially occurred with solids, which then progressed to soft foods but spared liquids.The patient reported a weight loss of 9.1 kg.An esophagogastroduodenoscopy was performed and she was subsequently diagnosed with pill esophagitis.At the same time, she was also diagnosed with oral LP, with no involvement of the esophagus.She was treated with a proton pump inhibitor that resolved her gastrointestinal symptoms.The symptoms returned one year later and a repeat esophagogastroduodenoscopy revealed white plaques due to LP.She was treated with intermittent glucocorticoids.Diagnosis of esophageal LP is crucial for the proper treatment.Some patients may require systemic immunosuppression and mechanical dilation to prevent weight loss.Surveillance endoscopies should be performed to monitor for squamous cell cancer.Cyclosporine has been used for genital and oral LP, but the present case is the first in which it has been used successfully to treat esophageal LP.

Second endoscopy
She was treated for candida esophagitis and underwent endoscopy three months later.Endoscopy was performed with a pediatric gastroscope under fluoroscopy and revealed strictures with white plaques and friable mucosa.

REvIEW
LP is a 'silent' disease until the patient develops strictures and experiences dysphagia.The following will describe the usual findings involved in LP esophagitis.

History and physical examination
Patients are usually asymptomatic; therefore, the incidence of esophageal LP is underestimated.Some patients may experience dysphagia due to strictures.Esophageal LP can also affect individuals with cutaneous LP -present as flat-topped, pruritic and violaceous papules or plaques in the flexural region of the arms, legs and wrists.Esophageal LP has a strong correlation to oral LP.Patients do not usually display symptoms of reflux disease.However, they are frequently mistaken to have gastroesophageal reflux disease and are treated without relief.

Endoscopic findings
Endoscopy reveals proximal stricture formation, ulcers, granulation tissue and inflammation in two-thirds of the esophagus.In addition, white, desquamating membranes with underlying friable mucosa can be found (Figure 5).The esophageal epithelium is usually atrophic and shows parakeratosis.

DISCuSSION
Early and accurate diagnosis of esophageal LP is crucial because there is a potential risk for malignant transformation.Early diagnosis also helps initiate appropriate treatment and saves the patient from unnecessary dilation and weight loss.According to Shenfine and Preston (2), the risk of malignant transformation in patients with oral LP is 1% to 3%, and 0.5% to 1% according to Abraham et al (3).The actual malignant risk of esophageal LP is unknown and may mirror that of oral LP.To date, only three cases of squamous cell carcinoma arising from esophageal LP have been reported (4,5).Esophageal LP is    under-reported because the symptoms are not always present.Dysphagia is common in symptomatic patients.LP tends to present in the proximal to mid esophagus.Because patients become uncomfortable when the endoscope approaches the epiglottis, there is a tendency to advance the endoscope quickly and miss oropharyngeal lesions, even by expert endoscopists.

Treatment
Steroid therapy is the key treatment.Dilation of the strictures is believed to cause more damage via the Koebner phenomenon.However, if the patient is symptomatic and steroids do not act quickly enough, dilation can be performed.Retinoic acid has also been recommended for esophageal LP.Tacrolimus has been shown to be beneficial for the treatment of esophageal LP (6).It is also used as a topical agent for the treatment of genital and oral LP.Cyclosporine has been used to treat genital LP (7).Antibacterial agents such as dapsone and immunosuppressants such as azathioprine have been used to treat only intractable cases of mucocutaneous LP (8,9).Unlike oral LP, esophageal LP cannot be treated with topical agents.Systemic agents are needed and relapse can occur once these agents are discontinued

CONCLuSION
Previous studies have shown that oral cyclosporine can be used successfully to treat oral LP (10).The present case is the first in which cyclosporine has been used successfully to treat esophageal LP.

Figure 2 )
Figure 2) Esophageal lichen planus with Candida species yeast infection

Figure 3 )
Figure 3) Barium swallow showing decreased luminal calibre throughout the entire esophagus

Figure 4 )
Figure 4) Dense lichenoid inflammatory infiltrate underneath the lamina propria due to lichen planus.Civatte bodies are eosinophilic hyaline spherical bodies that are seen beneath the epidermis, particularly in lichen planus