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Enteritis cystica profunda

M Kyriakos, S C Condon

    American Journal of Clinical Pathology
    |January 1, 1978
    PubMed

    Insights

    This study reports a novel case of "enteritis cystica profunda" in an adult with Peutz-Jeghers syndrome, a condition typically causing hamartomatous polyps. This finding suggests a potential link between Peutz-Jeghers syndrome and benign cystic lesions in the small intestine.

    Area of Science:

    • Gastroenterology
    • Pathology
    • Genetics

    Background:

    • Peutz-Jeghers syndrome is a rare genetic disorder characterized by hamartomatous polyps in the gastrointestinal tract.
    • Colitis cystica profunda (CCP) is a benign condition typically affecting the large intestine, presenting as cystic lesions.
    • Adenocarcinoma in Peutz-Jeghers syndrome is often associated with hamartomatous polyps.

    Observation:

    • A unique case of a patient with Peutz-Jeghers syndrome presented with ileal lesions histologically similar to colitis cystica profunda.
    • This represents the first reported adult case of such ileal lesions in conjunction with Peutz-Jeghers syndrome.
    • The authors propose the term "enteritis cystica profunda" for these small intestinal findings.

    Findings:

    • The histological features of the ileal lesions support their classification as "enteritis cystica profunda."
    • The association with Peutz-Jeghers syndrome suggests that some cases of colitis cystica profunda may also be hamartomatous in nature.
    • This benign condition might be misdiagnosed as small intestinal adenocarcinoma in some Peutz-Jeghers syndrome patients.

    Implications:

    • The proposed designation "enteritis cystica profunda" aids in characterizing these specific small intestinal lesions.
    • Recognizing this benign entity can prevent misdiagnosis of malignancy in Peutz-Jeghers syndrome patients.
    • This finding broadens the understanding of gastrointestinal manifestations associated with Peutz-Jeghers syndrome and cystic lesions.

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