[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases]
Tunisie Medicale [La]. 2008; 86 (11): 983-986
in French
| IMEMR
| ID: emr-119769
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
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Index:
IMEMR (Eastern Mediterranean)
Main subject:
Retrospective Studies
/
Ichthyosiform Erythroderma, Congenital
/
Ichthyosis
Limits:
Female
/
Humans
/
Male
Language:
French
Journal:
Tunisie Med.
Year:
2008
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