PMID: 9192409May 1, 1997Paper

Mal de Meleda: a report of four cases from the United Arab Emirates

Pediatric Dermatology
G G LestringantK I Qayed

Abstract

Mal de Meleda (MDM), or recessive transgressive palmoplantar keratoderma, is a rare disorder. MDM may have originated as a founder mutation that occurred on the island of Meleda, now Mljet, in Croatia, where it was first described. However, the condition has also been observed in countries distant from Mljet. The presentation of the disease in young patients has not been reported and the progressiveness of the lesions is debated. We examined four young United Arab Emirates nationals patients (ages 7 months to 12 years) who presented with keratoderma palmoplantaris (KPP) and transgressive pachyderma (TP) that had both been present before 1 year of age. KPP and TP were more pronounced in the two oldest patients. Family histories were consistent with autosomal recessive inheritance. The development of MDM lesions appears to be age-related. However, environment and individual factors may also play a role in the development and persistence of the lesions. Molecular genetic studies are necessary to establish whether the broad clinical presentation of the disease is due to allelic or genetic heterogeneities.

References

Jan 1, 1992·Dermatology : International Journal for Clinical and Investigative Dermatology·G G LestringantB J Blayney
May 1, 1989·International Journal of Dermatology·G G LestringantF Zagzouk
Aug 1, 1993·International Journal of Dermatology·V A ChotzenT M Mauro
Apr 1, 1996·Nature Genetics·X Estivill

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Citations

Oct 9, 2015·American Journal of Clinical Dermatology·Caroline Perez, Amor Khachemoune
May 12, 2001·The Journal of Investigative Dermatology·G G LestringantH C Hennies
Feb 6, 2004·Clinical Genetics·M MokniS Abdelhak
Nov 25, 2000·The Journal of Dermatology·T AymanE Yilmaz
Dec 16, 2011·Anais Brasileiros De Dermatologia·Fernanda Ayres de Morais e SilvaDenise Steiner
Dec 23, 2006·International Journal of Dermatology·Sheau-Chiou ChaoMei-Hui Yang
Dec 17, 2003·The British Journal of Dermatology·C CharfeddineS Abdelhak
May 18, 2021·Molecular and Cellular Biochemistry·Salim M A BastakiShreesh Ojha

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