Clinico-epidemiological features of congenital nonbullous ichthyosiform erythroderma in the eastern province of Saudi Arabia.

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.

Epidermolysis bullosa in the eastern province of Saudi Arabia.

BACKGROUND: Epidermolysis bullosa is recognized to be rare, but its prevalence in the Eastern Province of Saudi Arabia had not been previously established. METHODS: We reviewed 49,902 dermatology cases seen in our clinic over a 7-year period (1984 through 1990) and carried out a therapeutic trial of oral phenytoin in three severe cases of epidermolysis bullosa dystrophica (Recessive dystrophic type). RESULTS: Sixteen cases of epidermolysis bullosa were found in this series, ten (62.5%) of which were of the dermolytic type (epidermolysis bullosa dystrophica), and four (25%) of the epidermolytic type (epidermolysis bullosa simplex). Parental consanguinity was established in fourteen (87.5%) of these cases. All ten cases of epidermolysis bullosa dystrophica developed complications. Three severe cases were treated with oral phenytoin and managed with meticulous nursing and nutrition with blended foods and protein and vitamin supplements and responded satisfactorily. CONCLUSIONS: This study confirmed the rarity of epidermolysis bullosa in this province (population 3,000,000), and demonstrated the usefulness of oral phenytoin therapy, meticulous nursing, and good nutrition in the management of epidermolysis bullosa dystrophica.

Recessive dystrophic epidermolysis bullosa treated with phenytoin.

Three patients with severe recessive dystrophic epidermolysis bullosa were treated with oral phenytoin and palliative and supportive measures for variable periods. Their progress was compared with that of three milder cases managed only with palliative and supportive measures. The phenytoin-treated group showed marked decrease in blister count, increase in trauma tolerance, a rise in hemoglobin level, and considerable weight gain. The results support earlier reports that collagenase inhibitors are useful in controlling blister formation in recessive dystrophic epidermolysis bullosa.