TY - JOUR
T1 - Mal de Meleda
T2 - A report of four cases from the United Arab Emirates
AU - Lestringant, Gilles G.
AU - Frossard, Philippe M.
AU - Adeghate, Ernest
AU - Qayed, Khalil I.
PY - 1997/1/1
Y1 - 1997/1/1
N2 - 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.
AB - 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.
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U2 - 10.1111/j.1525-1470.1997.tb00234.x
DO - 10.1111/j.1525-1470.1997.tb00234.x
M3 - Article
C2 - 9192409
AN - SCOPUS:0031009031
SN - 0736-8046
VL - 14
SP - 186
EP - 191
JO - Pediatric Dermatology
JF - Pediatric Dermatology
IS - 3
ER -