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Dermatology

Peutz-Jeghers Syndrome

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1 Credit CME

JAMA Dermatology

Published Online: October 5, 2022

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Article Information and Disclosures

Eri Sato, MD¹;

Takao Goto, MD¹;

Hitoshi Honda, MD¹

Author Affiliations

¹Division of Infectious Diseases, Department of Medicine, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan

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A teenage girl was noted to have multiple dark-brown 1- to 2-mm hyperpigmented macules on the lips, nose (Figure), and conjunctivae that first appeared during infancy. At age 5 years she experienced intussusception that required an emergency laparotomy, which revealed gastrointestinal polyps. Histopathologic examination revealed the polyps to be hamartomatous, containing a tree-like proliferation of smooth muscle. There was no family history of hereditary hamartomatous polyposis syndromes. Given this constellation of features, a diagnosis of Peutz-Jeghers syndrome (PJS) was made. Genetic testing was not pursued given limited availability. She has required periodic colonoscopies to remove new polyps, and at last follow-up, pigmented cutaneous macules remained.

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Article Information

Corresponding Author: Eri Sato, MD, Tokyo Metropolitan Tama Medical Center, 2-8-29 Musashidai, Fuchu, Tokyo 183-8524, Japan (lilyfield310@gmail.com).

Published Online: October 5, 2022. doi:10.1001/jamadermatol.2022.3979

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References

Wendel D , Murray KF . Tumors of the digestive tract. In: Kliegman R , ed. Nelson Textbook of Pediatrics. 21st ed. Elsevier; 2020:2061-2064.e1.

Beggs AD , Latchford AR , Vanes HF , et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010;59(7):975-986.PubMed Google Scholar Crossref

Boland CR , Dios GE , Durno C , et al. Diagnosis and management of cancer risk in the gastrointestinal hamartomatous polyposis syndromes: recommendations from the US Multi-Society Task Force on Colorectal Cancer. Gastroenterology. 2022;162(7):2063-2085.PubMed Google Scholar Crossref

Remington BK , Remington TK . Treatment of facial lentigines in Peutz-Jeghers syndrome with an intense pulsed light source. Dermatol Surg. 2002;28(11):1079-1081.PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.