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Lelis Syndrome: A Rare Cause of Acanthosis Nigricans

Lelis Syndrome: A Rare Cause of Acanthosis Nigricans CORRESPONDENCE Pediatric Dermatology Vol. 33 No. 5 563, 2016 Hypodontia is seen in 75% of cases, and 62% of patients also have a furrowed tongue. Patients may LELIS SYNDROME: A RARE CAUSE OF also have a high arched palate. Yoshimura et al (6) ACANTHOSIS NIGRICANS recently reported successful treatment with acitretin To the Editor: of skin lesions seen in LS. A rare cause of acanthosis nigricans that is often LS is a unique cause of acanthosis nigricans. It overlooked is Lelis syndrome (LS), a unique clinical should be routinely included in the differential diag- syndrome characterized by ectodermal dysplasia and nosis in patients presenting with acanthosis nigricans acanthosis nigricans (1). Some authors have suggested and the above characteristic features. that LS occurs secondary to mutations in the EDA gene. The mode of inheritance is autosomal recessive. Van Steensel et al (2) have reported consanguinity in REFERENCES a few cases. 1. Guffey D, Narahari S, Alinia H. Hyperpigmented Palmoplantar hyperkeratosis is seen in approxi- plaques of the alar crease: an unusual presentation of mately 60% of individuals with LS. Accompanying acanthosis nigricans. Pediatr Dermatol 2016;33:e160– e161. hypohidrosis and hypotrichosis are seen in all patients 2. van Steensel M, Winnepenninckx V, Nagtzaam I et al. A with LS (3). Hyperconvex dystrophic nails (62%) are case of Lelis syndrome with hystrix-like ichthyosis. Am also frequently encountered. Eyelashes, especially the J Med Genet A 2008;146:2155–2158. lower ones, may be completely absent. Pubic and 3. Samdani AJ. Ectodermal dysplasia with acanthosis axillary hair are also absent in most patients. Acan- nigricans (Lelis’ syndrome). J Coll Physicians Surg Pak thosis nigricans is a characteristic feature of LS and is 2004;14:626–627. 4. van Steensel MA, van der Hout AH. Lelis syndrome may universally present in all patients with LS, usually be a manifestation of hypohidrotic ectodermal dysplasia. affecting the neck and the nose (4). Hystrix-like Am J Med Genet A 2009;149:1612–1613. ichthyosis may be seen, especially in the axillary 5. Steiner CE, Cintra ML, Marques-de-Faria AP. Ectoder- areas. Vitiligo is seen in 12.5% of patients. mal dysplasia with acanthosis nigricans (Lelis syndrome). Characteristic facial dysmorphism is usually pre- Am J Med Genet 2002;113:381–384. 6. Yoshimura AM, Velho PE, Magalh~aes RF et al. Lelis’ sent. Most patients have a long narrow face with up- syndrome: treatment with acitretin. Int J Dermatol slanting palpebral fissures. Midface hypoplasia is 2008;47:1330–1331. common. Strabismus and proptosis have also been SHAILENDRA KAPOOR, M.D. reported. Perioral radial furrows are seen in 88% of Private practice, Richmond, Virginia patients and perioral hyperpigmentation in 37.5%. e-mail: shailendrakapoor@yahoo.com Steiner et al (5) have reported prognathism too. © 2016 Wiley Periodicals, Inc. 563 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Dermatology Wiley

Lelis Syndrome: A Rare Cause of Acanthosis Nigricans

Pediatric Dermatology , Volume 33 (5) – Sep 1, 2016

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Publisher
Wiley
Copyright
Copyright © 2016 Wiley Periodicals, Inc.
ISSN
0736-8046
eISSN
1525-1470
DOI
10.1111/pde.12932
pmid
27595879
Publisher site
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Abstract

CORRESPONDENCE Pediatric Dermatology Vol. 33 No. 5 563, 2016 Hypodontia is seen in 75% of cases, and 62% of patients also have a furrowed tongue. Patients may LELIS SYNDROME: A RARE CAUSE OF also have a high arched palate. Yoshimura et al (6) ACANTHOSIS NIGRICANS recently reported successful treatment with acitretin To the Editor: of skin lesions seen in LS. A rare cause of acanthosis nigricans that is often LS is a unique cause of acanthosis nigricans. It overlooked is Lelis syndrome (LS), a unique clinical should be routinely included in the differential diag- syndrome characterized by ectodermal dysplasia and nosis in patients presenting with acanthosis nigricans acanthosis nigricans (1). Some authors have suggested and the above characteristic features. that LS occurs secondary to mutations in the EDA gene. The mode of inheritance is autosomal recessive. Van Steensel et al (2) have reported consanguinity in REFERENCES a few cases. 1. Guffey D, Narahari S, Alinia H. Hyperpigmented Palmoplantar hyperkeratosis is seen in approxi- plaques of the alar crease: an unusual presentation of mately 60% of individuals with LS. Accompanying acanthosis nigricans. Pediatr Dermatol 2016;33:e160– e161. hypohidrosis and hypotrichosis are seen in all patients 2. van Steensel M, Winnepenninckx V, Nagtzaam I et al. A with LS (3). Hyperconvex dystrophic nails (62%) are case of Lelis syndrome with hystrix-like ichthyosis. Am also frequently encountered. Eyelashes, especially the J Med Genet A 2008;146:2155–2158. lower ones, may be completely absent. Pubic and 3. Samdani AJ. Ectodermal dysplasia with acanthosis axillary hair are also absent in most patients. Acan- nigricans (Lelis’ syndrome). J Coll Physicians Surg Pak thosis nigricans is a characteristic feature of LS and is 2004;14:626–627. 4. van Steensel MA, van der Hout AH. Lelis syndrome may universally present in all patients with LS, usually be a manifestation of hypohidrotic ectodermal dysplasia. affecting the neck and the nose (4). Hystrix-like Am J Med Genet A 2009;149:1612–1613. ichthyosis may be seen, especially in the axillary 5. Steiner CE, Cintra ML, Marques-de-Faria AP. Ectoder- areas. Vitiligo is seen in 12.5% of patients. mal dysplasia with acanthosis nigricans (Lelis syndrome). Characteristic facial dysmorphism is usually pre- Am J Med Genet 2002;113:381–384. 6. Yoshimura AM, Velho PE, Magalh~aes RF et al. Lelis’ sent. Most patients have a long narrow face with up- syndrome: treatment with acitretin. Int J Dermatol slanting palpebral fissures. Midface hypoplasia is 2008;47:1330–1331. common. Strabismus and proptosis have also been SHAILENDRA KAPOOR, M.D. reported. Perioral radial furrows are seen in 88% of Private practice, Richmond, Virginia patients and perioral hyperpigmentation in 37.5%. e-mail: shailendrakapoor@yahoo.com Steiner et al (5) have reported prognathism too. © 2016 Wiley Periodicals, Inc. 563

Journal

Pediatric DermatologyWiley

Published: Sep 1, 2016

References