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Jonas Lelis and his syndrome
Khalid Al Aboud and Daifullah Al Aboud
Dermanities September 28, 2011; 7(1)
Figure.1:
John Lelis. This picture was taken in 2006.
Abstract:
Professor John Lelis is one of the Lithuanian pioneers in dermatology. In 1970s, Lelis described 4 unrelated patients of eastern European origin with the association of ectodermal dysplasia and acanthosis nigricans, a rare congenital genodermatosis that was later known as Lelis syndrome. This syndrome is characterized by hypotrichosis, hypohidrosis, and acanthosis nigricans. This report shed light on Lelis and the syndrome that bear his name.
Professor John Lelis (figure.1) is a Lithuanian world-renown professor of dermatology-venerology, one of the true pioneers in dermatology 1-2.Among his great contributions to dermatology, he is credited for describing a syndrome, that was later known as Lelis syndrome3-9.
Lelis syndrome (MIM ID %608290) also known as "Ectodermal dysplasia, hypohidrotic with acanthosis nigricans''9 is a rare autosomal recessive condition with only a few affected cases reported worldwide8.
It is characterized by hypotrichosis, hypohidrosis, and acanthosis nigricans. Additional features reported with this syndrome include: perioral radial furrows, hypodontia, palmoplantar hyperkeratosis, furrowed tongues, nail dystrophy, disturbances of skin pigmentation3 (perioral and periorbital hyperpigmentation, vitiligo, and perinevic leukoderma), and mentally retardation9.
The syndrome has been reported, so far, in Europe3, 6, 8, Saudi Arabia5 and Brazil3, 7. Van Steensel and van der Hout suggested that Lelis syndrome may be a manifestation of X-linked hypohidrotic ectodermal dysplasia.8.
Professor Jonas Lelis was born on July 5,19141,2, in Skaistgiris, Pušalotas district, Lithuania. In 1933, he finished Panevėžys secondary school for boys. In 1933-1939 he entered the Medical Faculty of Kaunas University named Vytauto Didžiojo. In1946, he went on studying at Moscow Central Institute of Dermatic and Venereal Diseases. Then he practiced medicine in Akmenė and Ylakiai, and was the head of local dispensaries. Later on he was a scientific worker at the Vilnius Institute of Dermatic and Venereal Diseases, the chief medical inspector at the Ministry for Medical Cure. From 1948-1993, he went on with his scientific career: he held the position lecturer at the Medical Faculty of Vilnius University, received the title of associate professor and professor2. He has written more than 300 scientific articles in Lithuanian and foreign journals, and 10 monographs of dermatology and venereology1. He was interested in many dermatological diseases but genetic skin diseases were one of his favorite topics. In 3 publications, he described1 the autosomally recessive inherited disease ectodermal dysplasia , so-called Lelis syndrome, in 7 patients of eastern European origin.
He published also fiction books and biographical essays1. In 1965 he became a republican prize winner for his Monograph “Red gum”2. His other monographs are widely known as well, e.g. “F.Šiaudinis – the discoverer of a bleak monster” (1971), “Inherited dermatosis and Syndromes” (1981), “The atlas of dermatic and venereal diseases” (written by Lelis and his co-author, 1998),“Tragical and comical miniatures” (1998), etc2. Jonas Lelis is a member of medical doctors’ corporation “Fraternitas Lituanica”. He is widely known for his high degree of intelligence and erudition.
Jonas Lelis is a polyglot with a good command of about ten foreign languages.
He has shared his knowledge lavishly with his students and doctors2. He is now 97 years old. He is still very bright and sharp, living with his daughter and her husband, in Lithuania.
Acknowledgment:
The author wish to thank the daughter of Prof Lelis, Rūta Paškauskienė and JURGITA STOŠKIENĖ from Era Esthetic laser dermatology clinic, Lithuania, for providing assistance in preparing this manuscript.
References:
1. Jonas Lelis. [A page on the Internet].From Wikipedia, the free encyclopedia Wikipedia® is a registered trademark of the Wikimedia Foundation, Inc . [This page was last modified 2011 April 5; cited 2011 April 5] .Available at; http://lt.wikipedia.org/wiki/Jonas_Lelis
2.Jonas Lelis.In, The signatories to the resumpted Hippocratic oaht in Lithuania in 1997. “Sveikatos mokslai” Nr.2, HEALTH SCIENCES. Page 837.
3.Lelis J. Autosomal recessive ectodermal dysplasia. Cutis. 1992 Jun; 49(6):435-7.
4.Steiner CE, Cintra ML, Marques-de-Faria AP. Ectodermal dysplasia with acanthosis nigricans (Lelis syndrome). Am J Med Genet. 2002 Dec 15; 113(4):381-4.
5. Samdani AJ. Ectodermal dysplasia with acanthosis nigricans (Lelis' syndrome). J Coll Physicians Surg Pak. 2004 Oct; 14(10):626-7.
6. van Steensel MA, Winnepenninckx V, Nagtzaam IF, Janssens R, De Vos R, Steijlen PM. A case of Lelis syndrome with hystrix-like ichthyosis. Am J Med Genet A. 2008 Aug 15; 146A (16):2155-8.
7. Yoshimura AM, Velho PE, Magalhăes RF, de Souza EM. Lelis' syndrome: treatment with acitretin. Int J Dermatol. 2008 Dec; 47(12):1330-1.
8. van Steensel MA, van der Hout AH. Lelis Syndrome may be a manifestation of hypohidrotic ectodermal dysplasia. Am J Med Genet A. 2009 Jul;149A(7):1612-3.
9. Lelis syndrome. [A page on the Internet].From OMIM, Online Mendelian Inheritance in Man . Copyright (c) 1966-2011 Johns Hopkins University [This page was last modified 2009 Dec 23; cited 2011 April 5] .Available at;http://lt.wikipedia.org/wiki/Jonas_Lelis