Primary hereditary ichthyoses in the Eastern Province of Saudi Arabia.

Abstract

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. To study and document the epidemiologic and demographic profiles of patients with PHI in the Eastern Province of Saudi Arabia. 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. 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%). This preliminary study has attempted to document the epidemiologic 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.