An interesting pill to swallow

Case

A 60-year-old woman presented with dysphagia and weight loss. Medical history was significant for oral and vulval lichen planus. Barium swallow and CT scanning revealed no abnormality. Oesophago-gastro-duodenoscopy demonstrated an ulcerated stricture in the upper oesophagus, 25 cm from the incisors (figure 1), with friable, exfoliating mucosa. Biopsies revealed chronic ulceration, degeneration of the basal epithelium, a dense junctional lymphocytic infiltrate and Civatte bodies (figure 2).

Figure 1.

Oesophageal stricture with ulcerated, friable mucosa with sloughing.

Figure 2.

Oesophageal biopsies showing lymphocytic inflammation with Civatte bodies.

Question

What abnormality is seen in the endoscopic image and histology slide? How is this condition managed?

Answer

A diagnosis of oesophageal lichen planus (OLP) was made. OLP is rare and under-recognised. Less than 100 cases have been reported worldwide.¹ Oesophageal LP has a strong female preponderance and is associated with oral LP in 89% cases.² Symptoms include dysphagia, in around 80% cases; odynophagia and weight loss are less common symptoms. Reflux-type symptoms, hoarseness, choking and epigastric pain can occur.^{1 2} Endoscopic findings are most common in the proximal and mid-oesophagus and range from superficial lesions to white papules, webs, pseudo-membranes and desquamation. Stricturing disease with a predilection for the proximal oesophagus is common. Differential diagnoses include reflux, eosinophilic and candida-oesophagitis. Histology typically shows a dense band-like lymphohistiocytic infiltrate in the lamina propria, basal-layer degeneration and necrotic keratinocytes (Civatte bodies).¹

Despite several balloon dilatations in conjunction with oral corticosteroids, our patient remained symptomatic. Intolerance to hydrochloroquine and azathioprine led us to the decision to start Mycophenolate Mofetil. She reported improvement in dysphagia symptoms and repeat oesophago-gastro-duodenoscopy 1 year later showed significantly improved appearances (figure 3). The patient remains in clinical and endoscopic remission at 5 years (figure 4).

Figure 3.

Endoscopic appearance of upper oesophagus after 1 year of treatment, with significantly less inflammation and stricturing.

Figure 4.

Endoscopic appearance of upper oesophagus with complete stricture resolution after 5 years of treatment.

There is no standardised medical treatment for OLP. Response rates to systemic corticosteroids are 74%; however, relapse rates are high (approximately 85%).³ Topical steroids provide short-lived symptom relief (up to 1 year).¹ Treatment response has also been reported with ciclosporin, tacrolimus and azathioprine.^{3 4} Endoscopic balloon dilatation can be used as an adjunct for patients who remain symptomatic despite medical therapy.³

We present a case of OLP with an unsatisfactory response to endoscopic therapy and oral corticosteroids, who demonstrated a sustained clinical and endoscopic response to mycophenolate mofetil at 5 years, sparing her from further balloon dilatation (and its concomitant risks). Our review of the literature revealed no reports of successful resolution of OLP with mycophenolate mofetil. We would like to propose its use in the maintenance of remission of OLP.

Ethics statements

Patient consent for publication

Obtained.