Abstract
Bullous congenital ichthyosiform erythroderma is a rare disorder of keratinization. It is associated with defective keratinization leading to skin fragility, blistering and hyperkeratosis. This condition was difficult to distinguish from staphylococcal scalded skin syndrome. We report a case of Bullous congenital ichthyosiform erythroderma in a 72 hour old male baby and discussed the differential diagnosis and management of the disease.
Highlights
Bullous congenital ichthyosiform erythroderma is a rare disorder of keratinization, it is known as epidermolytic hyperkeratosis [1]
Bullous congenital ichthyosiform erythroderma typically occurs in newborns they present with generalized erythroderma, characterized by erythema and scaling with or without edema[5]
There was history of bullous ichthyosiform disease in his sibling, physical examination showed, baby was afebrile, with multiple superficial erosions over the entire body and bullae over the hand has shown in the picture (2) fine scaly lesions was present over the face and chest as shown in the picture (3) and back
Summary
Bullous congenital ichthyosiform erythroderma is a rare disorder of keratinization, it is known as epidermolytic hyperkeratosis [1]. Bullous congenital ichthyosiform erythroderma is caused by mutations in keratin1 or keratin 10[3, 4]. These keratins are expressed in the differential spinous and granular layers of the epidermis, which are the sites of disease [2].
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