Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter To Editor
2008:74:2;154-155
doi: 10.4103/0378-6323.39705
PMID: 18388379

Peutz-Jeghers syndrome with prominent palmoplantar pigmentation

KN Shivaswamy, AL Shyamprasad, TK Sumathy, C Ranganathan
 Department of Dermatology and STD, M. S. Ramaiah Medical College, Bangalore, Karnataka, India

Correspondence Address:
K N Shivaswamy
Department of Dermatology and STD, M. S. Ramaiah Medical College, Bangalore - 560 054, Karnataka
India
How to cite this article:
Shivaswamy K N, Shyamprasad A L, Sumathy T K, Ranganathan C. Peutz-Jeghers syndrome with prominent palmoplantar pigmentation. Indian J Dermatol Venereol Leprol 2008;74:154-155
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology
Figure 2: Large hyperpigmented macules over the soles
Figure 2: Large hyperpigmented macules over the soles
Figure 1: Photograph showing lentigenes over the lips
Figure 1: Photograph showing lentigenes over the lips

Sir,

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by lentigines on the lips, buccal mucosa, tips of fingers and toes in association with intestinal polyps. PJS is caused by mutations in the gene coding for serine threonine kinase, located on the p arm of chromosome 19. [1],[2] The pigmented macules that usually appear during infancy and early childhood, are seen over the back of the hands and tips of the fingers and toes but are very rarely seen over the palms and soles. [3]

A 17-year-old male was referred from the surgery department to us for evaluation of the pigmented lesions over his palms and soles. Recently, he was operated for persistent colicky abdominal pain and was found to have an exophytic growth in the jejunum, which showed evidence of villous adenomas on histopathological examination. On enquiry, the patient gave a history of pigmented lesions over the palms and soles as well as the lips since childhood that increased in size and number over the past year. Two of his siblings also had history of pigmentation of the lips as well as their palms and soles. On examination, multiple discrete-to-coalescent, hyperpigmented macules 0.5-1.5 mm in size were seen over the lips [Figure - 1], perioral area and buccal mucosa. Similar hyperpigmented and round-to-oval macules of varying sizes ranging from 3 mm to more than 1 cm in diameter were present over the tips of the fingers and up to 5 cm in diameter were seen over the soles [Figure - 2]. A 12 cm-long paramedian linear scar was evident over the abdomen. A diagnosis of Peutz-Jeghers syndrome was made.

The pigmented macules of Peutz-Jeghers syndrome (PJS) usually appear during infancy and early childhood, but may be present at birth or develop later in life and have a tendency to increase in size during adolescence. Round, oval or irregular patches of brown or almost black pigmentation, 1-5 mm in diameter, are irregularly distributed over the buccal mucosa, gums, hard palate and lips, especially the lower lip. The pigmented macules may also occur on the face especially around the nose and the mouth but are smaller and often under 1 mm in size. Large macules are rarely seen over the back of the hands and the tips of the fingers and toes. [3] Our case had large hyperpigmented macules over both his palms and soles [Figure - 2], which is very unusual.

Intestinal manifestations include numerous polyps in the jejunum, ileum and less frequently in the colon, rectum, stomach and duodenum. Patients with PJS have a 10-18 fold greater lifetime cancer risk than the general population. The greatest risk is for gastrointestinal malignancy of the colon and duodenum. [4] Laugier-Hunziker syndrome and Cronkhite-Canada syndrome should be considered in the differential diagnosis of PJS. In the former, mucosal and nail pigmentation are characteristic but without intestinal polyps, whereas the volar aspects of the fingers are heavily pigmented along with evidence of intestinal polyps but without mucosal pigmentation in the latter. [5],[6]

Although perioral pigmentation is commonly seen in PJS, palmoplantar pigmentation is rare and such numerous large hyperpigmented macules over the soles have never been described before and hence, being reported.[7]

References
1.
Dormandy TL. Gastrointestinal polyposis with mucocutaneous pigmentation (Peutz-Jeghers syndrome). N Engl J Med 1949;241:993-1005.
[Google Scholar]
2.
Tsao H. Update on familial cancer syndromes and the skin. J Am Acad Dermatol 2000;42:939-46.
[Google Scholar]
3.
Bleehen SS, Anstery AV. Disorders of skin colour. In : Rook's text book of dermatology. Burns T, Breathnach S, Cox N, Griffiths C, editors. 7 th ed. Oxford: Blackwell Science; 2007. p. 1-39.68.
th ed. Oxford: Blackwell Science; 2007. p. 1-39.68.'>[Google Scholar]
4.
Disturbances of pigmentation. In : Andrew's diseases of the skin. Clinical Dermatology. James WD, Berger TG, Elston DM, editors. 10 th ed. Philadelphia: Saunders-Elsevier; 2006. p. 853-68.
th ed. Philadelphia: Saunders-Elsevier; 2006. p. 853-68.'>[Google Scholar]
5.
Veraldi S, Cavicchini S, Benelli C, Gasparini G. Laugier-Hunziker syndrome: A clinical, histopathologic and ultrastructural study of four cases and review of the literature. J Am Acad Dermatol 1991;25:632-6.
[Google Scholar]
6.
Herzberb AJ, Kaplan DL. Cronkhite-Canada syndrome. Int J Dermatol 1990;29:121-5.
[Google Scholar]
7.
Ortonne J, Bahadoran P, Fitzpatric TB, Mosher DB, Hori Y. Hypomelanosis and hypermelanosis. In : Fitzpatric's dermatology in general medicine. Freedberg IM, Eisener AZ, Wolff, et al , editors. 6 th ed, McGraw-Hill: 2003. p. 836-81.
et al , editors. 6 th ed, McGraw-Hill: 2003. p. 836-81.'>[Google Scholar]

Fulltext Views
227

PDF downloads
91
Show Sections