Epidermolytic Hyperkeratosis -NPS 2 Type

A case of bullous congenital ichthyosiform erythroderma (epidermolytic hyperkeratosis or EHK) with some unusual features is described. It was diagnosed in a 6 month old girl with no family history of either EHK or focal lesions suggestive of mosaicism. The girl probably had EHK type NPS-2; but non-involvement of face and lack of foul body odor set the case apart.