AZ Pediatría
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Vol. 25 - Num. 99

Clinical Reviews

Hyperpigmented macules and rectal prolapse, what should we suspect?

Pilar Ferrer Santosa, Diana Clavero Chuecaa, Carmen Navascués Cajalb, Irene Celiméndiz Ferrándezb, Manuel Lavilla Jiménezc

aPediatra. CS Tarazona. Zaragoza. España.
bEnfermera. CS Tarazona. Zaragoza. España.
cMédico de familia. CS Tarazona. Zaragoza. España.

Correspondence: P Ferrer . E-mail:

Reference of this article: Ferrer Santos P, Clavero Chueca D, Navascués Cajal C, Celiméndiz Ferrández I, Lavilla Jiménez M. Hyperpigmented macules and rectal prolapse, what should we suspect? . Rev Pediatr Aten Primaria. 2023;25:273-6.

Published in Internet: 15-09-2023 - Visits: 2000


Peutz-Jeghers syndrome (PJS) is an autosomal dominant syndrome with an incidence of 1 in 200,000 live births. The most frequent clinical manifestations are hyperpigmented macules typically located on the oral mucosa and the presence of polyposis in the gastrointestinal tract.

Unlike adulthood, in pediatrics the development of malignant tumor pathology is exceptional. However, in the pediatric age group, a high index of suspicion must be maintained when faced with a patient diagnosed with PJS who presents with abdominal pain compatible with intussusception, since this complication is relatively frequent and requires urgent surgical treatment. Once the diagnosis of this disease has been made, periodic controls should be carried out by means of endoscopies starting at eight years of age.


Hyperpigmentation skin Peutz-Jeghers Syndrome Polyposis Rectal prolapse


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