Document Detail


Clinico-epidemiological features of primary hereditary ichthyoses in the Eastern province of Saudi Arabia.
MedLine Citation:
PMID:  16533225     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: A total of 10 455 new dermatology patients were seen in the dermatology clinics of King Fahad Hospital of the University (KFHU), Al-Khobar, Saudi Arabia, between January 1990 and December 1995. We identified 71 patients with a histopathologically confirmed diagnosis of specific forms of primary hereditary ichthyoses (PHI). We have reviewed the epidemiological and clinical features of these patients. OBJECTIVE: To document the epidemiological and clinical features of patients with PHI in the Eastern Province of Saudi Arabia. METHODS: We used the dermatology out-patient department (OPD) logbooks to identify cases of PHI from new patients presenting with different dermatological problems over a 6-year period. We used specifically designed protocol forms to extract epidemiological and clinical data from the study patients' medical records. These were entered into a computer database and analyzed using standard statistical software. RESULTS: A total of 71 patients, 44 males and 27 females (male : female sex ratio of 1.63 : 1), were identified from a total of 10 455 new patients seen in our dermatology clinics between January 1990 and December 1995. The occurrence rate of PHI, in our clinics, was 0.67% or 7 per 1000 new dermatology cases. The clinical pattern of PHI showed that Icthyosis Vulgaris was the most common form seen, followed by Nonbullous Ichthyosiform Erythroderma. Thirty-one patients (44.7%) with Ichthyosis Vulgaris (IV) were seen: 12 patients (16.9%) with X-linked Recessive Ichthyosis (XLRI), four patients (5.6%) with Lamellar Ichthyosis (LI), three patients (4.2%) with Bullous Ichthyosiform Erythroderma (BIE) and 21 patients (29.6%) with Nonbullous Ichthyosiform Erythroderma (NBIE). There was a significantly high consanguinity rate in our patients (85%), and the family history was positive in 53 cases out of the 71 (75%). CONCLUSIONS: This preliminary study is the first report of its kind from Saudi Arabia and documents the clinico-epidemiological features of PHI patients in the Eastern Province. The high rate of parental consanguinity among our Saudi patients may account for the high proportion of patients with a positive family history. Consanguinity also probably explains why the most severe forms of PHI were seen in a significant number of new patients' siblings. These severe forms of PHI (Nonbullous Ichthyosiform Erythroderma, Bullous Ichthyosiform Erythroderma and Lamellar Ichthyosis) together constituted nearly 40% of all cases.
Authors:
Ahmad A Al-Zayir; Omar M Al-Amro Al-Alakloby
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  International journal of dermatology     Volume:  45     ISSN:  0011-9059     ISO Abbreviation:  Int. J. Dermatol.     Publication Date:  2006 Mar 
Date Detail:
Created Date:  2006-03-14     Completed Date:  2006-07-06     Revised Date:  2006-10-30    
Medline Journal Info:
Nlm Unique ID:  0243704     Medline TA:  Int J Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  257-64     Citation Subset:  IM    
Affiliation:
Department of Dermatology, College of Medicine, King Faisal University, Dammam, Saudi Arabia. alzayir@yahoo.co.uk
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MeSH Terms
Descriptor/Qualifier:
Age of Onset
Consanguinity*
Female
Genetic Diseases, Inborn / epidemiology*
Genetic Predisposition to Disease
Health Surveys
Humans
Ichthyosis / epidemiology*
Incidence
Infant
Infant, Newborn
Male
Retrospective Studies
Saudi Arabia / epidemiology
Comments/Corrections
Erratum In:
Int J Dermatol. 2006 Apr;45(4):488

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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