Mal de Meleda: A Report of Four Cases from the United Arab Emirates

: 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...

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Bibliographic Details
Published in:Pediatric dermatology 1997-05, Vol.14 (3), p.186-191
Main Authors: Lestringant, Gilles G., Frossard, Philippe M., Adeghate, Ernest, Qayed, Khalil I.
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Child, Preschool
Dermatology
Dyskeratosis
Female
Humans
Infant
Keratoderma, Palmoplantar - epidemiology
Keratoderma, Palmoplantar - pathology
Male
Medical sciences
Skin - pathology
Tropical medicine
United Arab Emirates - epidemiology
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Summary:: 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.
ISSN:0736-8046
1525-1470
DOI:10.1111/j.1525-1470.1997.tb00234.x