Document Detail

Clinico-epidemiological features of congenital nonbullous ichthyosiform erythroderma in the eastern province of Saudi Arabia.
MedLine Citation:
PMID:  15482290     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: A total of 10 455 new dermatology patients were seen in the dermatology clinics of King Fahd Hospital of the University (KFHU), Al-Khobar, Eastern Saudi Arabia, between January 1990 and December 1995. We identified 21 patients with a histopathologically confirmed diagnosis of congenital nonbullous ichthyosiform erythroderma (CNBIE). We have reviewed the epidemiological and clinical features of these patients. OBJECTIVE: To document the epidemiological and clinical features of patients with CNBIE in eastern Saudi Arabia. METHODS: We used the dermatology outpatient department (OPD) logbooks to identify diagnosed cases of CNBIE from new patients presenting with different dermatological problems over a 6-year period. We used specifically designed data-collection protocol forms to extract epidemiological and clinical data from the patients' medical records. These were entered into a computer database and analysed using standard statistical software. RESULTS: A total of 21 patients (five males, 16 females) with a male : female ratio of 0.31 : 1 were identified from a total of 10 455 new patients seen in our dermatology clinics over the study period. The occurrence rate of CNBIE in our clinics was 0.2%, or two per 1000 new dermatology cases. Nineteen (90%) of 21 CNBIE patients were born with collodion membranes. Eighty-one per cent of our patients had a positive family history of CNBIE. Consanguinity among the parents of our CNBIE patients was significantly high at 95%. CONCLUSIONS: To the best of our knowledge, this preliminary study is the first report of its kind from Saudi Arabia (documenting the clinico-epidemiological features of CNBIE patients in the Eastern Province). The high rate of parental consanguinity among the parents of our Saudi CNBIE patients may account for the high incidence rate of this genodermatosis in eastern Saudi Arabia. In comparison with results of other studies that reported a low occurrence rate of CNBIE among dermatology patients, our results were of a significantly higher rate.
O M Al-Amro Al-Akloby; A A Al-Zayir
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the European Academy of Dermatology and Venereology : JEADV     Volume:  18     ISSN:  0926-9959     ISO Abbreviation:  J Eur Acad Dermatol Venereol     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-10-14     Completed Date:  2005-02-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9216037     Medline TA:  J Eur Acad Dermatol Venereol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  659-64     Citation Subset:  IM    
Department of Dermatology, College of Medicine, King Faisal University, Dammam, Saudi Arabia.
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MeSH Terms
Data Collection / methods
Ichthyosis, Lamellar / diagnosis,  epidemiology*
Infant, Newborn
Saudi Arabia / epidemiology

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