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

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.

Clinical and Genetic Studies of 3 Large, Consanguineous, Algerian Families With Mal de Meleda

“…Progressivity of the lesions was seen in all our patients except the youngest; this has been reported previously in a series of patients from Tunisia 7 and the United Arab Emirates, 8 where an age-related progression in the extension of the lesions was mentioned.…”

“…This probably reflects individual variation in the presentation of the disease. [6][7][8] The main histological findings in our patients with MdM were marked orthokeratosis or parakeratosis and hypergranulosis.…”

“…Hyperhidrosis, perioral erythema, and lichenoid plaques are also noted. [6][7][8] The evolution of this disorder often exhibits a progressive character.…”

“…Since the first description of the disorder in 1826 in the islands off the coast of Dalmatia, other cases have been reported from Europe, [23][24][25][26][27][28] Africa, 7 Asia, 6,8,29,30 and America. 31 This report adds 14 cases from Algeria.…”

Mutations of a country: a mutation review of single gene disorders in the United Arab Emirates (UAE)

“…Variability in clinical features has been described. This condition is seen frequently in the UAE [Lestringant et al, 1992b[Lestringant et al, , 1997 and molecular study [Eckl et al, 2003] in a UAE Bedouin family showed a homozygous mutation affecting the translation initiation codon of SLURP1 gene (Table 8).…”