Lamellar ichthyosis in a Saudi kindred.
A series of 20 patients aged 4-16 years presented with lamellar ichthyosis at the dermatology unit of King Faisal Hospital at Taif in western Saudi Arabia. Though they had come from different families, they all belonged to the same tribe that was confined to a rural area in the precincts of Taif. The tribe is known for consanguineous marriages. The affected children are from 5 sibships. All the children presented with generalized thick dark scales. The history revealed that they all were born with a thick membrane around them, which was shed soon after. Gradually over months and years there was development of generalized scaling. A prototype is shown in the Figure. The scales had become darker and thicker with the passage of time. There was no pruritus. Teeth and mucous membranes were normal. None of the patients had erythroderma. The associated findings in some of the children were ectropion and alopecia. Contractures including pseudoainhum were also observed in some children. None of the patients' parents was found to be affected with a similar condition. There was no improvement with age. Skin biopsies from several of the patients revealed only hyperkeratosis. All had to be managed with topical emollients since they could not afford oral retinoids.
Al Aboud
Daifullah
D
Dermatology Department, King Khalid Military City Hospital, Hafer Al Baten, Saudi Arabia.
Al Aboud
Khalid
K
Ramesh
V
V
Kumar
Joginder
J
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006801:Humans; D017490:Ichthyosis, Lamellar / Q000235:genetics*; D010641:Phenotype; D012529:Saudi Arabia