[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases]

ABSTRACT

Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms. To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital Tunisian study. A retrospective study of all cases of ichihyosis referred during a period of 5 years to the department of dermatology of Charles Nicole's hospital of Tunis. Sixty cases of hereditary ichihyosis were seen. The sex-ratio was of 0.5. Parental consanguinity was noted in 36 patients [60%]. Seventeen patients [25.7%] had a positive familial history of ichthyosis. The clinical form of ichthyosis was determined in 52 cases. The nonbullous ichthyosiform erythroderma was observed in 25 patients [41.6%]. Sixty patients presented an ichihyosis vulgaris [26.6%]. The other forms of ichthyosis were rarely observed 4 cases of X-linked recessive ichthyosis, 2 cases of lamellar ichthyosis and 2 cases of bullous iclithyosiform erythroderma. Two patients were born with collodion-like membranes. Two cases presented a complex syndrome. The NBIE, commonly considered as a rare form of ichthyosis, was the most frequently form seen in our study [41.6%], probably because of the high frequency of consanguineous marriages in Tunisia. The IV represents the most frequent form reported in the litterature and was observed in 25% of our patients. The classification of some ichthyosis associated with other extra-cutaneous abnormalities [found in 2 of our patients] remains difficult