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The aberrant intraepithelial T cells in refractory coeliac disease show epidermotropism
  1. Hongxiang Liu1,
  2. Rishi K Fofaria2,
  3. Simon M Greenfield2,
  4. Alistair Robson3,
  5. Penny Wright1,
  6. Peter G Isaacson4,
  7. Jeremy Woodward5,
  8. Ming-Qing Du1,6
  1. 1 Department of Histopathology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  2. 2 Department of Gastroenterology, Queen Elizabeth II Hospital, Welwyn Garden City, Hertfordshire, UK
  3. 3 St John's Institute of Dermatology, St Thomas’ Hospital, London, UK
  4. 4 Department of Cellular Pathology, The Royal Free Hospital, London, UK
  5. 5 Department of Gastroenterology and Clinical Nutrition, Addenbrooke's Hospital, Cambridge, UK
  6. 6 Division of Molecular Histopathology, Department of Pathology, University of Cambridge, Cambridge, UK
  1. Correspondence to Professor Ming-Qing Du, Division of Molecular Histopathology, Department of Pathology, University of Cambridge, Level 3 Lab Block, Box 231, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK; mqd20{at}cam.ac.uk

https://doi.org/10.1136/jclinpath-2013-202066

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    Coeliac disease is a gluten-mediated autoimmune disorder that occurs in genetically predisposed individuals carrying the HLA-DQ2 and/or HLA-DQ8 alleles. Most patients with coeliac disease can be effectively managed by a strict gluten-free diet. Approximately, 5% of adult patients with coeliac disease lose their clinical and histological response to a strict gluten-free diet and develop refractory coeliac disease (RCD). Based on the clonal and immunophenotypical features of intraepithelial lymphocytes (IELs), RCD is divided into two subtypes. In RCD-I, IELs are polyclonal and show a normal phenotype, while in RCD-II, IELs are monoclonal and immunophenotypically aberrant, expressing cytoplasmic CD3ε but not surface CD3ε, CD8 and T cell receptor (TCR) antigens.1–3

    Coeliac disease mainly causes enteropathy of the small intestine, but can involve the entire gastrointestinal tract. Apart from the classic intestinal syndrome, patients with coeliac disease also show a wide spectrum of extraintestinal manifestations and 10–20% of patients with coeliac disease present with dermatitis herpetiformis. Here, we report a case of RCD with subsequent presentation of skin lesions and show that the neoplastic intraepithelial T cells of the gut also preferentially invade the epidermis.

    The patient, a 47-year-old man, presented with a 2-year history of epigastric pain, occasional nausea and vomiting, intermittent light and bulky stool, and weight loss. Oesophagogastroduodenoscopy showed erosion and inflammation in the body and antrum of the stomach and proximal duodenum, and mild erythema in the distal duodenum. Campylobacter-like organism (CLO) test …

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    Footnotes

    • Contributors HL collected the experimental data. RKF, SMG and JW provided clinical input. AR, PW, PGI and M-QD reviewed the histology and immunohistochemistry. M-QD analysed the data and drafted the manuscript. All authors reviewed and commented on the manuscript and approved its submission.

    • Funding The research in M-QD lab was supported by grants from Leukaemia & Lymphoma Research, UK, the Kay Kendal Leukaemia Fund, UK.

    • Competing interests None.

    • Patient consent Obtained.

    • Ethics approval Oxfordshire REC A.

    • Provenance and peer review Not commissioned; externally peer reviewed.