DYSCHROMATOSIS UNIVERSALIS HEREDITARIA A CLINICO-EPIDEMIOLOGICAL STUDY FOR TWELVE CASES IN AL-RAMADI CITY
*Abdullah Mancy [FICMS (D&V)] and Khalid Mohammad Awad [FICMS (D&V)]
ABSTRACT
Background: Dyschromatosis universalis hereditaria is uncommon hereditary disease usually has autosomal dominant inheritance, its pathogenesis is unknown, characterized by generalized asymptomatic hyperpigmented and hypopigmented macules on the trunk and extremities in a mottled appearance. Objective: To shed light on the prevalence and clinical presentation of dyschromatosis universalis hereditaria in Iraqi patients. Patients and Methods: Patients presenting with features of dyschromatosis universalis hereditaria were studied with their families within period from 2008-2012 in dermatology department in AL-Ramadi Teaching Hospital which serving a population around 0.8 million. In all patients a detailed history was taken and careful physical examination of all patients, regarding skin and other systems were carried out. Results: Twelve patients with dyschromatosis universalis hereditaria, six females and six males were studied, their ages ranged from 10 years -54 years old with a mean age 30 years, their age of onset ranged from birth - 15 years with a mean age of onset was 6.25 years. The prevalence was 15/106, the inheritance was autosomal recessive in 83.3%, Consanguinity of patient’s parents was present in 91.7%, the cutaneous manifestations were the main complaint which affects the sun protected areas mainly, histopathological examination revealed focal increased melanin deposition within the keratinocytes of the basal cell layer. Conclusion: This study had confirmed that dyschromatosis universalis hereditaria is common disease and to remind clinicians about diagnosis of the disease should be kept in mind for patients presenting with generalized mottled dyspigmentation.
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