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

Abstract

BACKGROUND:

Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms.

AIM:

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.

CONCLUSION:

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.

• Authors: Monia Kharfi, Nadia El Fekih, Donia Ammar, Aida Khaled, Bécima Fazaa, Mohamed Ridha Kamoun
• Journal: La Tunisie medicale (Tunis Med) Vol. 86 Issue 11 Pg. 983-6 (Nov 2008) ISSN: 0041-4131 [Print] Tunisia
• Vernacular Title: Ichtyoses herediatires en Tunisie: etude epidemiologique a propos de 60 cas.
• PMID: 19213489 (Publication Type: English Abstract, Journal Article)

Topics

• Adolescent
• Age of Onset
• Child
• Child, Preschool
• Consanguinity
• Female
• Genes, Recessive
• Genetic Diseases, Inborn (epidemiology, genetics, pathology)
• Genetic Predisposition to Disease
• Humans
• Ichthyosis (epidemiology, genetics, pathology)
• Ichthyosis, Lamellar (epidemiology, genetics)
• Incidence
• Male
• Pedigree
• Retrospective Studies
• Tunisia (epidemiology)