The majority of keratinization disorders are referred to as Mendelian disorders of
cornification. This is a very broad group, clinically characterized by hyperkeratosis or visible …

Inherited ichthyoses, defined as the generalized form of Mendelian disorders of cornification,
are characterized by visible scaling and/or hyperkeratosis of most or all of the skin. This …

Hereditary palmoplantar keratodermas (PPK s) comprise a large and heterogeneous group
of disorders characterized by persistent thickening of the epidermis at palmar and plantar …

Hereditary palmoplantar keratodermas (PPK) comprise a clinically and genetically
heterogeneous group of genodermatoses, which share impaired epidermal differentiation …

Disorders of cornification are a group of diseases that share abnormalities in the
manufacture or desquamation of corneocytes. This paper reviews the major and a few of the …

The keratodermas inherited in an autosomal dominant way are a heterogeneous group of
disorders characterized by thickening of the palms and soles due to abnormal keratinization …

The palmoplantar skin is a highly specialized tissue which is able to resist mechanical
trauma and other physical stress. In recent years the more descriptive classification of …

Mendelian Disorders of Cornification (MEDOC): The Ichthyoses Page 1 121.1 CHAPTER 121
Mendelian Disorders of Cornification ( MEDOC): the Ichthyoses Daniel Hohl 1 & Mary Williams 2 …

Hereditary palmoplantar keratodermas (PPK) comprise a clinically and genetically
heterogeneous group of genodermatoses, which share the characteristic of impaired …

Background and Design: Epidermolytic hyperkeratosis (EHK) is a rare autosomal dominant
disorder of cornification. While different clinical presentations of EHK have been described …