Endoscopy 2019; 51(02): E26-E27
DOI: 10.1055/a-0767-6204
E-Videos
© Georg Thieme Verlag KG Stuttgart · New York

Langerhans cell histiocytosis: a rare case of large-bowel inflammatory lesions

Beatrice Marinoni
1  Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Federica Cavalcoli
1  Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Alessandro Del Gobbo
2  Pathology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Helmut Neumann
3  First Medical Department, University Medical Center Mainz, Mainz, Germany
,
Emanuela Passoni
4  Dermatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Maurizio Vecchi
1  Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
5  Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Gian Eugenio Tontini
1  Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
› Author Affiliations
Further Information

Corresponding author

Beatrice Marinoni, MD
Gastroenterology and Endoscopy Unit
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico
Via della Commenda 12
20122 Milan
Italy   
Fax: +39-02-55033414   

Publication History

Publication Date:
23 November 2018 (eFirst)

 

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal neoplastic proliferation of Langerhans cells, with typical expression of CD1a, S100 protein, and langerin [1] [2]. Gastrointestinal involvement is extremely rare and usually observed in systemic illness with poor prognosis and high morbidity [2] [3] [4].

Here, we report the case of a 48-year old man affected by mucocutaneous Langerhans disease, presenting with severe weight loss, constipation, and nausea. He was previously diagnosed with LCH with pituitary localization in 2002 and underwent treatment with vinblastine, steroids, and mercaptopurine. An esophagogastroduodenoscopy was unremarkable, including duodenal and gastric biopsies. Ileocolonoscopy was performed using a high definition colonoscope with optical enhancement (OE mode) virtual chromoendoscopy (Optivista EPK-i7010 video processor; Pentax, Tokyo, Japan) [5].

During withdrawal, the OE mode 2 was activated to increase the detection of subtle mucosal changes, revealing multiple yellowish nodules of 5 – 10 mm in size, with central erosions or aphthous ulcers ([Video 1]). OE mode 1 showed both the pit and vascular patterns to be clearly characterized as nonadenomatous (Kudo II) and irregular or interrupted ([Fig. 1], [Video 1]).

Video 1 Ileocolonoscopy showing multiple sites of Langerhans cell histiocytosis with high definition optical enhancement (OE mode) chromoendoscopy. OE1 – 2 mode showed detailed image of a nodule with nonadenomatous pit pattern (Kudo II), interrupted vascular pattern, and aphthous ulcer.

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Zoom Image
Fig. 1 High definition endoscopic image with optical enhancement (OE) mode 1, showing a nodule of 8 mm with nonadenomatous pit pattern (Kudo II), interrupted vascular pattern, and central aphthous ulcer.

Histology on targeted biopsy samples demonstrated submucosal proliferation of histiocytes, with abundant eosinophilic cytoplasm, positive anti-CD1a, anti-CD68 R, and S100 protein immunohistochemistry, which are pathognomonic for colonic localization of LCH ([Fig. 2]).

Zoom Image
Fig. 2 Histology of targeted endoscopic biopsies. a Hematoxylin and eosin stain. Diagnostic immunohistochemistry: b anti-CD1a; c anti-CD68R; d S100 protein.

This case is relevant for several reasons. First, we describe an unusual case of gastrointestinal LCH, which unconventionally manifested with symptoms that warranted endoscopic investigations. In addition, we provide for the first time detailed video and image documentation of intestinal LCH to further support clinicians and endoscopists during their everyday practice.

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Competing interests

None


Corresponding author

Beatrice Marinoni, MD
Gastroenterology and Endoscopy Unit
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico
Via della Commenda 12
20122 Milan
Italy   
Fax: +39-02-55033414   


Zoom Image
Fig. 1 High definition endoscopic image with optical enhancement (OE) mode 1, showing a nodule of 8 mm with nonadenomatous pit pattern (Kudo II), interrupted vascular pattern, and central aphthous ulcer.
Zoom Image
Fig. 2 Histology of targeted endoscopic biopsies. a Hematoxylin and eosin stain. Diagnostic immunohistochemistry: b anti-CD1a; c anti-CD68R; d S100 protein.