Harold Jeghers

Biography

• Born 26 September 1904, Jersey City, USA
• 1928 – Graduated with a Bachelor of Science in biology from the Rensselaer Polytechnic Institute, Troy, New York
• 1932 – Graduated medicine from the Case Western Reserve University, Cleveland, Ohio
• 1932-36 – Commenced internship and progressed to house officer at Fifth Medical Service, Boston City Hospital
• 1936 – Consultant physician at Boston City Hospital
• 1942 – Associate professor of medicine and physician in chief at Boston City Hospital
• 1946 – Professor of medicine and Chief Physician at Georgetown University School of Medicine
• 1956 – Professor of medicine at Seton Hall College
• 1966 – Director of medicine at St. Vincent Hospital, Worcester, Massachusetts
• 1974 – Retired from professorships
• 1976 – Consultant for the Cleveland Health Sciences Library, Case Western Reserve University
• Died 21 September 1990, Massachusetts, USA

Medical Eponyms

Peutz-Jeghers syndrome

Peutz-Jeghers syndrome is an autosomal dominant condition caused by mutations in the serine/threonine kinase 11 gene (STK11/LKB1) is characterised by melanotic macules; gastrointestinal hamartomatous polyps; and increased cancer risk.

1939 – Jeghers saw two patients with melanin pigmentation associated with intestinal polyposis, which he believed to be part of a syndrome:

A 14-year old American schoolgirl who presented to the Fifth Medical Service of Boston City Hospital with 6 weeks of persistent watery non-bloody diarrhoea 5-6 times per day. Her ileum was resected in 1933 following the second episode of intussusception and intestinal obstruction, which found polyps of the stomach, ileum and sigmoid. The patient had numerous small, brown-black spots around the mouth, lips and oral mucous membrane, in addition to the dorsal fingers and toes. The spots on the lips were present since early childhood according to the parents.

A 39-year old Italian-French woman who presented with a protruding rectal mass, and 9 months of constipation and intermittent rectal bleeding. She had previously been admitted with abdominal pain, nausea and vomiting. Numerous brown and bluish-brown pigmented spots on the face, eyes, mouth, lips, gums, fingers and toes were noted. Multiple small and large intestine polyps were seen on gastrointestinal x-rays. Ileostomy, partial colectomy, fulguration of polyps in the rectosigmoid and anastomosis between the ileum and rectosigmoid were performed; however, the patient subsequently died of intraperitoneal and wound infection. Autopsy demonstrated polyps in the stomach, duodenum, jejunum, ileum, and rectum, with a brown-gray ‘fish-skin’ appearing small intestine mucosa.

1944 – Jeghers published these two cases in his three-part NEJM review article of skin discolouration and causes, titled ‘Pigmentation of the Skin‘. He provided a brief literature review of two similar cases with skin pigmentation and polyposis reported by Hutchinson in 1896 (later confirmed to have intussusception by Weber in 1919), and Metzger et al. (1935)

1949 – Jegher, McKusick, and Katz published a case series of 10 patients with intestinal polyposis and melanin spots of the oral mucosa, lips and digits (including the two cases above) and proposed it as a distinct new syndrome. They provided a literature review of other similar cases, including reports by Peutz (1921) and Touraine & Couder (1945).

Major Publications

• Jeghers HR, Houghton JD, Foley JA. Weil’s disease: Report of a Case with Postmortem Observations and Review of Recent Literature. Archives of Pathology. 1935;20:447–476.
• Jeghers H. Vitalizing medical education in hospitals. Hospital Progress. 1954;35:60.
• Jeghers H. Medical care education and research: Philosophy and technics of self education for the medical student and physician. New Eng J Med. 1964;271:1297–1301.
• Jeghers H. Pigmentation of skin. New Eng J Med. 1944;231:88–100, 122–136, and 181–189. [Peutz-Jeghers syndrome – case reports of 2 patients]
• Jeghers H, McKusick VA, Katz KH. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits; a syndrome of diagnostic significance. N Engl J Med. 1949;241(25): 993-1005. [Peutz-Jeghers syndrome – case series of 10 patients]
• Jeghers H, McKusick VA, Katz KH. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits; a syndrome of diagnostic significance. N Engl J Med. 1949;241(26):1031-1036. [Peutz-Jeghers syndrome – literature review]

References

Biography

• Beighton P, Beighton G. Harold Joseph Jeghers (1904 – 1990). In: The Man Behind the Syndrome. Springer 1991:214.
• Bury YA. Jeghers, Harold Joseph (1904–1990). In: van Krieken J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer 2016.
• Bibliography. Jeghers, Harold. WorldCat Identities

Eponymous terms

• Hutchinson J. Pigmentation of lip and mouth. Archives of Surgery, 1896; 7: 290. [Plate CXLI]
• Weber FP. Patches of deep pigmentation of oral mucous membrane not connected with Addison’s disease. Quart J Med. 1919;12:404-408.
• Metzger IL, Ohlmann J, and llalff M. Un cas de polyposc du colon. Arch d mal de Vapp digestif. 1935;25:90-95.
• Peutz JLA. Very remarkable case of familial polyposis of mucous membrane of intestinal tract and nasopharynx accompanied by peculiar pigmentations of skin and mucous membrane. Nederl maandschr v geneesk. 1921;10:134–146.
• Touraine A, Couder F. Syndrome de Peutz (Lentigino–polypose digestive). Ann de dermat et syph. 1945;5:313.
• Keller JJ, Offerhaus GJ, Giardiello FM, Menko FH. Jan Peutz, Harold Jeghers and a remarkable combination of polyposis and pigmentation of the skin and mucous membranes. Fam Cancer. 2001;1(3-4):181-185.