نتایج جستجو برای: keratinization disorders
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62 Indian Dermatology Online Journal | Volume 9 | Issue 1 | January‐February 2018 Sir, Darier disease [DD; Darier‐White disease or (dys) keratosis follicularis], is an autosomal dominant disorder of keratinization with characteristic dermatologic findings such as keratotic papules on the seborrheic regions, brittle nails and longitudinal erythronychia, and palmoplantar pitting. Hypopigmented ma...
Ichthyosis is defined as a group of diseases with keratinization disorder and diffuse scaling with highly variable degree of involvement. According to our knowledge, coincidence of ichthyosis and dermatophytosis, which both are very common disorders, is a very rare event. We report a young man with congenital ichthyosis that histological analysis of his skin biopsies and direct smear revealed P...
Sir, Bullous congenital ichthyosiform erythroderma (BCIE), or epidermolytic hyperkeratosis (MIM# 113.800), is a rare disorder of keratinization associated with blistering in its early phase. It was first described by Jean-Louis Brocq in 1902 as érythrodermie congénitale ichthyosiforme avec hyperépidermotrophie. Although most cases are sporadic, familial cases show an autosomal dominant pattern ...
Palmoplantar keratoderma (PPK) is a heterogeneous group of hereditary and acquired disorders characterized by abnormal thickening of the palms and soles. There are three clinical patterns: diffuse, focal, and punctuate. Palmoplantar keratodermas can be divided into the following functional subgroups: disturbed gene functions in structural proteins (keratins), cornified envelope (loricrin, trans...
Porokeratoses are a group of different entities that belong to the skin keratinization disorders. From the histological point of view the main and common characteristic of these disorders is the presence of compact parakeratotic columns known as cornoid lamellae. All varieties should be carefully treated and followed-up because of the risk of developing malignant epithelial tumors. We report th...
Keratodermas encompass a wide spectrum of disorders of keratinization that may be acquired or hereditary. We present two cases of focal acral hyperkeratosis (FAH), a subtype of punctate palmoplantar keratoderma. We review the literature and attempt to clarify the confusing classification of the heritable punctate palmoplantar keratodermas.
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