A case of Cronkhite-Canada syndrome with repeated linked color imaging observation of the subepithelial capillary network in the colon.


Journal

Clinical journal of gastroenterology
ISSN: 1865-7265
Titre abrégé: Clin J Gastroenterol
Pays: Japan
ID NLM: 101477246

Informations de publication

Date de publication:
Oct 2022
Historique:
received: 15 04 2022
accepted: 12 07 2022
pubmed: 22 7 2022
medline: 28 9 2022
entrez: 21 7 2022
Statut: ppublish

Résumé

Cronkhite-Canada syndrome (CCS) is a non-hereditary disorder characterized by non-neoplastic gastrointestinal polyposis and ectodermal changes. While corticosteroids are considered effective, some cases are refractory. A 48-year-old woman presented with diarrhea, anorexia, and epigastralgia lasting for 3 months. She suffered from alopecia and nail dystrophy. Gastrointestinal endoscopy with histological examination confirmed non-neoplastic polyposis from the stomach to the rectum, confirming the diagnosis of CCS. Linked color imaging (LCI) with magnified endoscopy revealed a ribbon-like proliferation of capillaries surrounding the pits in the colonic mucosa. Histologically, the polyps had dilated glands, edematous stroma with inflammatory cell infiltrates and increased capillaries just beneath the epithelium. Immunohistochemical examination confirmed the expression of vascular endothelial growth factor (VEGF), mainly in the superficial epithelial and crypt cells. Steroid therapy was ineffective, and concomitant infliximab therapy provided symptomatic relief. Although symptoms rapidly improved with combination therapy, capillary hyperplasia and slight inflammation persisted in the colon mucosa after polyp resolution. Withdrawal of steroid treatment resulted in flare-ups of symptoms and polyps. Repeated magnified observations at LCI during post-relapse retreatment clearly captured the resolution process of both neovascularization and inflammation. Once the capillary hyperplasia and inflammation subsided, the steroid could be tapered off without relapse. To our knowledge, this is the first report describing the involvement of VEGF-induced angiogenesis and LCI findings in CCS; LCI observations are useful not only in the active phase of CCS, but also in determining subtle capillary hyperplasia and residual inflammation in remission, which may be an indicator of continued treatment.

Identifiants

pubmed: 35864388
doi: 10.1007/s12328-022-01678-6
pii: 10.1007/s12328-022-01678-6
doi:

Substances chimiques

Vascular Endothelial Growth Factor A 0
Infliximab B72HH48FLU

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

934-940

Informations de copyright

© 2022. Japanese Society of Gastroenterology.

Références

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Auteurs

Yayoi Matsumoto (Y)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Fumikazu Koyama (F)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan. fkoyama@naramed-u.ac.jp.
Division of Endoscopy, Nara Medical University Hospital, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan. fkoyama@naramed-u.ac.jp.

Kohei Morita (K)

Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Hiroyuki Kuge (H)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Shinsaku Obara (S)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.
Division of Endoscopy, Nara Medical University Hospital, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Yosuke Iwasa (Y)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Takeshi Takei (T)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Tomomi Sadamitsu (T)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Chiho Ohbayashi (C)

Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Masayuki Sho (M)

Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

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