[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases].
BACKGROUND
Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms.


OBJECTIVE
To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital tunisian study.


METHODS
A retrospective study of all cases of ichthyosis referred during a period of 5 years to the department of dermatology of Charles Nicole's hospital of Tunis.


RESULTS
Sixty cases of hereditary ichthyosis 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 ichthyosis 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 ichthyosiform erythroderma. Two patients were born with collodion-like membranes. Two cases presented a complex syndrome.


CONCLUSIONS
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 literature and was observed in 25% of our patients. The classification of some ichthyosis associated with other extracutaneous abnormalities (found in 2 of our patients) remains difficult.
Kharfi
Monia
M
Service de Dermatologie, Hôpital Charles Nicole, Tunis, Tunisie.
El Fekih
Nadia
N
Ammar
Donia
D
Khaled
Aida
A
Fazaa
Bécima
B
Ridha Kamoun
Mohamed
M
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D005808:Genes, Recessive; D030342:Genetic Diseases, Inborn / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D007057:Ichthyosis / Q000453:epidemiology* / Q000235:genetics* / Q000473:pathology; D017490:Ichthyosis, Lamellar / Q000453:epidemiology / Q000235:genetics; D015994:Incidence; D008297:Male; D010375:Pedigree; D012189:Retrospective Studies; D014416:Tunisia / Q000453:epidemiology