Clinical and Epidemiological Features of 19 Cases of Congenital Icthyosiform Erythroderma in Northern Nigeria: A case series
Keywords:
Congenital Icthyosiform Erythroderma, Genodermatosis, Abnormal keratinization, Collodion membrane, Scaly skinAbstract
Congenital Icthyosiform Erythroderma (CIE) is a rare genetically related skin disease (genodermatosis) that
is characterized by abnormal keratinization of the skin. The disease is a variant of autosomal recessive
congenital icthyosis that presents with collodion membrane at birth, generalized erythroderma and fine white
scaling of the skin. It is a lifelong disease that is associated with psychological, physical, emotional and
financial burden to the patients and their families. It is a rare disease with a prevalence of 1/200000 to
1/1000000 million birth. High incidence of consanguinity has been reported in pedigrees of CIE and this may
play a role in transmission of the recessive defective genes causing the disease. We present a case of 19
family members with CIE associated with high rate of consanguineous marriage among the family members.
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