Iraqi Postgraduate Medical Journal

Abstract

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 from
April 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 pigmentosa
CONCLUSION:
Genodermatoses are frequently encountered among Iraqi dermatological outpatients and more common in families with positive consanguinity and were comparable to other countries