ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
EC23 — Genetic disorders of skin pigmentation
EC23.1 — Syndromic genetically-determined hypermelanosis or...
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:
- Polyposis syndrome #2669
- Inherited cancer-predisposing syndromes #2829
- Genetic or developmental disorders involving lips or oral mucosa #13302
- Polyposis syndrome #14141
- Polyposis syndrome #14174
- Genetic or developmental disorders involving lips or oral mucosa #15229
- Syndromic genetically-determined hypermelanosis or lentiginosis #16277
- Genetic or developmental disorders involving lips or oral mucosa #16873
- Genetic or developmental disorders involving lips or oral mucosa #22812
- Phakomatoses or hamartoneoplastic syndromes #23395