Jonas Lelis and the syndrome that bears his name

Khalid Al Aboud, Mohammad Hakim, Ibrahim Alehibi, Homaid Alotaibi, Samar Alwafi

Dermatology Department, King Faisal Hospital, Makkah, Saudi Arabia

Corresponding author: Khalid Al Aboud, MD

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How to cite this article: Al Aboud K, Hakim M, Alehibi I, Alotaibi H, Alwafi S. Jonas Lelis and the syndrome that bears his name. Our Dermatol Online. 2021;12(4):473-475.

Submission: 11.10.2020; Acceptance: 15.12.2020

DOI: 10.7241/ourd.20214.34

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© Our Dermatology Online 2021. No commercial re-use. See rights and permissions. Published by Our Dermatology Online.

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ABSTRACT

Jonas Lelis (1914 – 2011) was a professor and one of the Lithuanian pioneers in dermatology. In the 1970s, Lelis described four unrelated patients of eastern European origin with an association of ectodermal dysplasia and acanthosis nigricans, a rare congenital genodermatosis that was to be known as Lelis syndrome. The syndrome is characterized by hypotrichosis, hypohidrosis, and acanthosis nigricans. This report sheds light on Jonas Lelis and the syndrome that bears his name.

Key words: Acanthosis nigricans; Ectodermal dysplasia; Lelis syndrome

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Jonas Lelis (Fig. 1) was a world-renown Lithuanian professor of dermatology and venerology and a pioneer in dermatology [1–3]. Among his great contributions to dermatology, he is credited for describing a syndrome that was to be known as Lelis syndrome (LS) [4–10].

Figure 1: Jonas Lelis (1914–2011).

Lelis syndrome (MIM ID %608290), also known as Ectodermal dysplasia and hypohidrosis with acanthosis nigricans [10], is a rare autosomal recessive condition with only several cases reported worldwide [9].

It is characterized by hypotrichosis, hypohidrosis, and acanthosis nigricans. Additional features reported include perioral radial furrowing, hypodontia, palmoplantar hyperkeratosis, a furrowed tongue, nail dystrophy, disturbances in skin pigmentation [4] (perioral and periorbital hyperpigmentation, vitiligo, and perinevic leukoderma), and mental retardation [10].

Some authors have suggested that LS occurs secondary to mutations in the EDA gene [2].

The syndrome has, so far, been reported in Europe [4,7,9], Saudi Arabia [6], and Brazil [4,8]. Van Steensel et al. suggested that Lelis syndrome may be a manifestation of X-linked hypohidrotic ectodermal dysplasia [9].

Some authors reported successful treatment with acitretin of skin lesions seen in LS.8

Jonas Lelis was born on July 5, 1914 [1,3], in Skaistgirys, Pušalotas District, Lithuania. In 1933, he finished Panev?žys Secondary School for Boys. Between 1933 and 1939, he attended the Medical Institute at Kaunas University, named after Vytautas Magnus. After graduating from Kaunas University in 1938, J. Lelis practiced medicine in Šakiai, Panev?žys, Akmen? and Ylakiai. In the years 1946-1957, he worked at the Vilnius Institute of Dermatological and Venereal Diseases, since 1948 as a director; and he also was the chief medical inspector in the Ministry.. From 1948 through 1993, he continued his scientific career. He held the position of a lecturer at the Medical Faculty of Vilnius University and received the title of associate professor and professor [3]. He had written more than 300 scientific articles in both Lithuanian and foreign journals and 10 monographs on dermatology and venereology [1]. He had developed interest in numerous dermatological diseases, but genetic skin diseases were one of his favorites.

In three of his publications, he described an autosomal recessive disease, ectodermal dysplasia (Lelis syndrome), in seven patients of eastern European origin. He had also published fiction books and biographical essays [1]. In 1965, he become a republican prize winner for his monograph “Red gum” [3]. His other monographs are widely known as well, to name a few, “F. Schaudinn: The discoverer of the pale monster” (on Fritz R. Schaudinn as a scientist of Lithuanian roots) (1971), “Inherited dermatoses and syndromes” (1981), “The atlas of dermatological and venereal diseases” (by Lelis and a co-author, 1998), and “Tragical and comical miniatures” (1998) [3]. Lelis was a member of the medical corporation Fraternitas Lituanica. He is widely known for a high degree of intelligence and erudition. He did sports, played chess, and was a polyglot with a good command of about ten foreign languages. He had shared his knowledge lavishly with his students and doctors alike [3]. Lelis died at the age of 97 on December 31, 2011.

ACKNOWLEDGEMENTS

The authors wish to thank the daughter of Jonas Lelis, R?ta Paškauskien?, and Jurgita Stoskiene from a laser dermatology clinic in Lithuania for providing assistance in preparing this article.

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 2019 June 27;cited 2020 Oct 5]. Available at;https://lt.wikipedia.org/wiki/Jonas_Lelis

2. Kapoor S. Lelis syndrome:A rare cause of acanthosis nigricans. Pediatr Dermatol. 2016;33:563.

3. Jonas Lelis. In, The signatories to the resumpted Hippocratic oath in Lithuania in 1997. “Sveikatos mokslai“Nr.2, Health Sciences. Page 837.

4. Lelis J. Autosomal recessive ectodermal dysplasia. Cutis. 1992;49:435-7.

5. Steiner CE, Cintra ML, Marques-de-Faria AP. Ectodermal dysplasia with acanthosis nigricans (Lelis syndrome). Am J Med Genet. 2002;113:381-4.

6. Samdani AJ. Ectodermal dysplasia with acanthosis nigricans (Lelis’syndrome). J Coll Physicians Surg Pak. 2004;14:626-7.

7. 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;146A:2155-8.

8. Yoshimura AM, Velho PE, Magalhães RF, de Souza EM. Lelis’syndrome:Treatment with acitretin. Int J Dermatol. 2008;47:1330-1.

9. van Steensel MA, van der Hout AH. Lelis Syndrome may be a manifestation of hypohidrotic ectodermal dysplasia. Am J Med Genet A. 2009;149A:1612-3.

10. Lelis syndrome. [A page on the Internet]. From OMIM, Online Mendelian Inheritance in Man. Copyright (c) 1966-2020 Johns Hopkins University [This page was last modified 2009 Dec 4;cited 2020 Oct 5]. Available at;https://www.omim.org/entry/608290

Notes

Source of Support: Nil,

Conflict of Interest: None declared.