Transcript Slide 1 - K.f.u.p.m ISI

Primary Hereditary Ichthyoses In The Eastern Province Of Saud
Arabia
Al-Zayir, AA; Al-Amro Alakloby, OM
BLACKWELL PUBLISHING LTD, INTERNATIONAL JOURNAL OF DERMATOLOGY;
pp: 415-419; Vol: 43
King Fahd University of Petroleum & Minerals
http://www.kfupm.edu.sa
Summary
Background Of 10,455 new dermatology patients seen at the Dermatology Clinic of
King Fahad Hospital of the University, Al-Khobar, Saudi Arabia, between January
1990 and December 1995, the epidemiologic and demographic features of 71 patients,
each with a histopathologically confirmed diagnosis of one specific type of primary
hereditary ichthyosis (PHI), have been reviewed. Objective To study and document
the epidemiologic and demographic profiles of patients with PHI in the Eastern
Province of Saudi Arabia. Methods From the outpatient department dermatology
logbooks, 71 patients with PHI, from 10,455 new cases with different dermatologic
problems seen during the 5-year period, were included in this study. Using specially
designed protocol forms, epidemiologic and demographic data were extracted from
the medical records of the patients, entered into a computer, and analyzed using the
SPSS program. Results Seventy-one patients with PHI, 44 males and 27 females
(ratio, 1.63 : 1), were seen at our Dermatology Clinic between January 1990 and
December 1995. The occurrence rate of PHI in our clinic is 0.67%, i.e. approximately
7 per 1000 new cases. The clinical pattern of PHI was as follows: 31 patients (44.7%)
with ichthyosis vulgaris (IV), 12 (16.9%) with X-linked recessive ichthyosis (XLRI),
four (5.6%) with lamellar ichthyosis (LI), three (4.2%) with bullous ichthyosiform
erythroderma (BIE), and 21 (29.6%) with nonbullous ichthyosiform erythroderma
(NBIE). IV was most common, followed by NBIE. Parental consanguinity was high
(approximately 85%), and the family history was positive in 53 of the 71 cases (75%).
Conclusions This preliminary study has attempted to document the epidemiologic
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Copyright: King Fahd University of Petroleum & Minerals;
http://www.kfupm.edu.sa
patterns of PHI patients in Saudi Arabia and, to our knowledge, this is the first report
of its kind in this country. We found a high rate of parental consanguinity, a high
percentage of patients with a positive family history, and many siblings affected by
the severest forms of PHI, namely NBIE, BIE, and LI, which accounted for 39.4% of
all cases of PHI.
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Copyright: King Fahd University of Petroleum & Minerals;
http://www.kfupm.edu.sa