non-bullous congenital ichthyosiform erythroderma
Also found in: Dictionary, Acronyms, Encyclopedia.
non-bullous congenital ichthyosiform erythrodermaA non-bullous ichthyosis (OMIM:242100)—a skin disorder characterised by abnormal cornification of the epidermis—that is milder than lamellar ichthyosis and has a greater variability in intensity of erythema, size and type of scales. Here, the scales are usually white, fine and powdery, and accompanied by palmoplantar keratoderma, often with painful fissures, digital contractures and loss of pulp volume.
Defects of TGM1, which encodes a transglutaminase, are a cause of nonbullous congenital ichthyosiform erythroderma.