Pérez AR

Language: Spanish
References: 6
Page: 122-125
PDF size: 171.57 Kb.

ABSTRACT

Lamellar ichthyosis or congenital ichthyosiform erythroderma is a rare autosomal recessive genodermatosis (1 out of 300 000 births in the world) characterized by erythroderma, palmoplantar keratoderma, onychomycosis and other disorders. A case of a 42-year-old white woman who was born with reddened skin, later becoming dry and scaly and who developed hair loss, teeth abnormalities, tearing and ectropion is presented. This patient attended consultation belatedly, at the age of 13 years. In addition, maternal family history of the same disease was confirmed. The clinical diagnosis of lamellar ichthyosis was established based on the gathered evidence. A comprehensive treatment aimed at genetic counselling, symptomatic management for her skin care and consultation with other specialties for multidisciplinary care was indicated to the patient and her mother.

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