“…In the normal process of keratinization, profilaggrin, contained within keratohyalin granules, is broken down to filaggrin, which acts as a matrix for the aggregation of keratin filaments and is associated with the degradation of keratohyalin granules. 1,2,5,7,8 Metze and Rü tten 2 demonstrated by immunoelectron microscopy that the cytoplasm of granular parakeratotic cells was unreactive for filaggrin. This provides evidence that a defect in the processing of filaggrin probably occurs in this disorder.…”