ICD-11 classes
20 Developmental anomalies
Multiple developmental anomalies or syndromes
LD2D — Phakomatoses or hamartoneoplastic syndromes
LD2D.0 — Peutz-Jeghers syndrome

ICD-11 LD2D.0 — Peutz-Jeghers syndrome

Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterised by intestinal hamartomatous polyps in association with a distinct pattern of skin and mucosal macular melanin deposition. Patients have an increased risk of developing intestinal cancer.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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