Porokeratosis is an inherited, clonal disorder of epidermal keratinization classically characterized by development of single or multiple annular lesions with raised, sharply marginated, keratotic ridge and central atrophy. This clinical presentation may vary with different types of porokeratosis but histopathologically unified by the consistent finding of ‘cornoid lamella’. Porokeratosis may d...

The discovery, in 2006, that loss-of-function mutations in the filaggrin (FLG) gene are the cause of ichthyosis vulgaris-the most common disorder of keratinization-and also a strong genetic risk factor for atopic eczema, marked a significant breakthrough in the understanding of eczema pathogenesis. Subsequent investigations of the role of FLG-null mutations have identified a series of significa...

Porokeratosis (PK) is an uncommon disorder of epidermal keratinization characterized by annular plaques with an atrophic center surrounded by a raised, keratotic wall, with unknown aetiology and an unpredictable outcome. It has several clinical forms including porokeratosis of Mibelli, giant porokeratosis, linear porokeratosis, disseminated superficial actinic porokeratosis, palmoplantar poroke...

Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules primarily affecting seborrheic areas on the head, neck, thorax, and less frequently the oral mucosa. When oral manifestations are p...

Terra firma-forme dermatosis (TFFD) is a bizarre, acquired and idiopathic dermatosis that etiology has still not been fully defined. It is characterized by the presence of asymptomatic, brownish dirt-like lesion maybe due to disorder of keratinization. These lesions cannot be removed with ordinary cleansing. Therefore, TFFD can be differentiated from dermatosis neglecta. Patient was a 17-year-o...

Acrokeratosis verruciformis of Hopf is a rare genodermatosis with an autosomal dominant mode of inheritance. It is a disorder of keratinization, characterized by multiple, flat-topped, skin-colored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet. Histopathologically, the lesion shows considerable hyperkeratosis, acanthosis, and papillomatosis, mim...

Sites of synthesis of sulfur-containing protein in keratinizing epithelium have been studied by radioautography after injection of sulfur-labeled cystine. Bern et al. (3) demonstrated concentration of silver grains immediately below the cornified layer, suggesting that much of sulfur is added at the final stages of keratinization as observed in hair. In contrast, B6langer (4) reported that prot...

287 Indian Dermatology Online Journal | Volume 8 | Issue 4 | July‐August 2017 Sir, We read with great interest the informative case report “Pachyonychia congenita with late onset (PC tarda)” by Sravanthi et al. in the July‐August 2016 issue of the Journal.[1] However, we would like to emphasize certain points regarding the history and current classification system of this rare disorder of kerat...

The Gulf Journal of Dermatology and Venereology INTRODUCTION The term acne is derived from Greek word “acme” meaning “prime of life”. Though considered as a benign disorder, it can have profound psychological impact on the patients, especially with those having disfiguring scars, which can persist for life.1 It is a chronic inflammatory disorder of pilosebaceous unit resulting from increased se...