Frequency of Genodermatoses Among Iraqi Patients


ABSTRACT:BACKGROUND:Genodermatoses are hereditary skin disorders or anomalies which can be grouped into three categories: chromosomal, single gene and multifactorial. Most genodermatoses show single gene or Mendelian inheritance (autosomal dominant, autosomal recessive or X-linked recessive genes).OBJECTIVE:To asses the frequency of genodermatoses among Iraqi patients in outpatients Dermatology and Venereology comparison with other countries.PATIENTS AND METHODS:This case series descriptive epidemiological study included eighty three patients (57males and 26 females) with genodermatoses. They consulted the out patient clinic/ Department of Dermatology and Venereology Baghdad Teaching Hospital fromApril 2005 through April 2006. Their ages ranged from 2months-60 years (Median 10 years),With various genetic diseases.Full history, dermatological and clinical examinations were done to establish the clinical diagnosis of genodermatoses regarding all demographic points related to these disorders.RESULTS:The frequency of genodermatoses among outpatient attendant in Dermatology and Venereology Department was 83/ 20000 (0.42%).This study had shown that the most common genodermatoses were; ichthyosis: 21 (25.3 %) patients and epidermolysis bullosa which contain 16 (19.3 %) patients when taken together they constituted 37 (44.6%) patients of the total, neurofibromatosis 8 (9.6 %), hereditary palmoplantar keratoderma 6 (7.2%), darier's disease 5 (6 %) and xeroderma pigmentosa 4 (4.8 %).Positive family history of the same disease was obtained in; 8 (38.1 %) patients with ichthyosis, 4 (66.6 %) in hereditary palmoplantar keratoderma, 2 (12.5 %) in epidermolysis bullosa and all patients with Hailey-Hailey disease had positive family history of the same condition.Consanguinity was positive in; 13 (61.9 %) patients of ichthyosis, 12 (75 %) epidermolysis bullosa, 2 (33.3 %) hereditary palmoplantar keratoderma and (100 %) patients with xeroderma pigmentosaCONCLUSION:Genodermatoses are frequently encountered among Iraqi dermatological outpatients and more common in families with positive consanguinity and were comparable to other countries