A case at the crossroads of dermatology and gastroenterology

February 08, 2018 By: Bjorn Rembacken

A case at the crossroads of dermatology and gastroenterology

What next for a middle-aged patient with a condition affecting her skin and mouth? 

Several years ago, a middle-aged woman presented with a condition affecting her skin (photograph A) and mouth (photograph B), and she was diagnosed with lichen planus.

The patient then presented with dysphagia. A lesion was found high in the oesophagus (photograph C) and biopsy samples were taken (photograph D).

 

Case question 1

WHAT IS THE AETIOLOGY OF THE STRICTURE?

a) Benign

b) Malignant 

Answer to case question 1, discussion and case question 2

Answer to case question 1

Correct answer: A.

 

Discussion

Lichen planus is a condition of unknown aetiology involving the skin, nails and often the mucous membranes. Classically, skin lesions are shiny, violaceous, flat papules (as seen in photograph A) that are mainly found on the front of the wrists, the lumbar region and around the ankles.  Mucous membrane lesions complicate 30–70% of cases and consist of white plaques (as shown in photograph B), erosions and, rarely, ulceration inside the mouth or on the tongue, genitalia or anus. 

Oesophageal involvement of lichen planus has probably been under-reported.1  Dickens et al.2 reported oesophageal involvement in 5/19 patients and a more recent study by Kern et al. found it in 20/32 patients.3 Oesophageal involvement usually develops in patients who have oral lesions. Indeed, the pathological appearance of lichen planus is similar in the mouth and in the oesophagus, with a band-like lymphocytic infiltrate including ‘Civatte bodies’,4 a nonspecific feature of interface dermatitis that is particularly common on lichen planus.  Macroscopically, the oesophageal appearance can include peeling of the friable squamous mucosa, white plaques, ulcers, erosions and strictures,4 and typically affects the proximal oesophagus.

Cutaneous lichen planus is a self-limiting disease, with spontaneous regression of skin lesions within 1 to 2 years; however, both oral and oesophageal lichen planus tend to be chronic.  A review from the Mayo clinic reported good results for treatment with topical steroids, fluticasone 880 mcg twice daily or budesonide 3 mg twice daily, with two thirds of patients responding well.5  Of course, any stricturing is managed with dilatations. Dilatations in the absence of medical therapy have been associated with exacerbation of non-oesophageal disease in a Köbner-like phenomenon.6

 

Case question 2

The stricture in the case patient was successfully managed with repeated dilatations over a 10-year period. However, she again developed dysphagia and another lesion was found at endoscopy (photograph E). Once more, biopsy samples were obtained (photograph F).

 

 

WHAT IS THE DIAGNOSIS NOW?

a)     Lichen planus

b)     Candida

c)     Squamous dysplasia

d)     Squamous cell carcinoma

Answer to case question 2 and discussion

Answer to case question 2

Correct answer: D.

 

Discussion

This oesophageal lesion looks very different to how it appeared previously. There is now a nodularity of the surface mucosa with irregular vessels and a brown discoloration seen on narrow-band imaging (NBI). This is a neoplastic lesion and the nodularity with central ulceration suggests that the lesion is invasive rather than in situ. 

Malignant changes to the oesophagus have been reported in up to 10% of patients who have oesophageal involvement of lichen planus.7 Indeed, a large population-based study from Finland confirmed that lichen planus is associated with a markedly increased risk of cancer of the lip (SIR 5.17, 95% CI 3.06–8.16), tongue (SIR 12.4, 95% CI 9.45–16.00 and oral cavity (SIR 7.97, 95% CI 6.79–9.24).  However, the risk of oesophageal cancer was more moderately increased (SIR 1.95, 95% CI 1.17–3.04).8 

As the lesion was small, and both a CT scan and EUS were reassuring, our cancer team decided to attempt endoscopic resection of the lesion. The ultimate histological specimen is shown in photograph G and shows the presence of lymphovascular invasion. The patient subsequently underwent chemoradiotherapy with curative intent.

 

 

About the author

Bjorn Rembacken is at Leeds Teaching Hospitals NHS Trust, Leeds, UK. He was born in Sweden and qualified from Leicester University in 1987. He undertook his postgraduate education in Leicester and in Leeds. His MD was dedicated to inflammatory bowel disease. Dr Rembacken was appointed Consultant Gastroenterologist, Honorary Lecturer at Leeds University and Endoscopy Training Lead in 2005. Follow Bjorn on Twitter @Bjorn_Rembacken

https://twitter.com/Bjorn_Rembacken

 

Comments

Ahmed Gabr, February 17, 2018 21:20
b
Dmitry Haurilenka, February 17, 2018 11:25
C
Ana Bran, February 12, 2018 20:30
b
Jurij Hanzel, February 08, 2018 16:55
B - oesophageal SCC in the setting of lichen planus

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