Brunauer-Fuhs-Siemens palmoplantar keratoderma, commonly known as striate palmoplantar keratoderma, is a rare, autosomally inherited disease of linear hyperkeratosis in which patient usually presents with conspicuous longitudinal hyperkeratosis on volar surface of hands and feet. Mutations in 3 genes namely desmoglein 1, desmoplakin and keratin 1, have been identified and held responsible for t...

We investigated whether or not the topographic regulation of melanocyte differentiation is determined by mesenchymal-epithelial interactions via fibroblast-derived factors. The melanocyte density in palmoplantar human skin (i.e., skin on the palms and the soles) is five times lower than that found in nonpalmoplantar sites. Palmoplantar fibroblasts significantly suppressed the growth and pigment...

In this study, a possible new syndrome affecting 18 members of a family spanning 4 generations is described. The main features include palmoplantar hyperkeratosis, proportionate short stature, facial dysmorphism, clinodactyly, epilepsy, deafness, and hypodontia. This syndrome is inherited in an autosomal dominant manner with a high degree of penetrance but variable expressivity. This syndrome d...

Aquaporin-5 (AQP-5) is a water-transporting protein expressed in mammal sweat glands. It has been reported that the expression of AQP-5 is involved in sweating of mice, rats, and horses. However, the physiological function of human AQP-5 is still uncertain. In this report, we examined the expression pattern of AQP-5 in the skin lesions of palmoplantar hyperhidrosis in patients with Nagashima-ty...

the inheritance of olmsted syndrome that is a very rare congenital with transgredient palmoplantar keratoderma is distinguished by the presence of massive hyperkeratosis with fissured skin and periorificial chaps. it usually appears during the early life and mostly in male pateints. herein we report a case of olmsted syndrome which is associated with ichthyosis and somatic type of delusion duri...

eczema is a very common disorder that affects children. to find out the national prevalence of eczema in iranian children, we conducted a systematic review and meta-analysis. we conducted a literature review by using the google scholar, pubmed in medline area, altavista, iranmedex and magiran in august 2013. the search terms included: prevalence, eczema, children, pediatric, allergy, isaac and ...

Background: Dermatoses affecting the palms and soles are more common in our day-to-day practice, because they most frequently exposed to various allergens, mechanical stress infectious agents than any other parts body. Apart from diagnostic difficulties, few palmoplantar dermatoses cause great discomfort disability may also affect a persons livelihood. Aim:To study clinical features frequency o...

M utations in connexin 26 (GJB2, Cx26) cause autosomal recessive and occasionally dominant non-syndromic sensorineural hearing loss (SNHL). Cx26 mutations have also been identified in SNHL with dermatological features of autosomal dominant diffuse palmoplantar hyperkeratosis (DPPK). We describe a girl with bilateral sloping sensorineural hearing loss, striate palmoplantar hyperkeratosis (SPPK),...

BACKGROUND An autosomal dominant form of diffuse non-epidermolytic palmoplantar keratoderma, palmoplantar keratoderma of Bothnian type, is caused by mutations in the AQP5 gene encoding the cell-membrane water channel protein aquaporin 5 leading to defective epidermal-water-barrier function in the epidermis of the palms and soles. CASE PRESENTATION We report the first Danish family diagnosed w...

A multicentre case-control study of 216 patients with palmoplantar pustulosis and 626 controls with miscellaneous dermatoses showed a considerably higher prevalence of smoking in the group with palmoplantar pustulosis. This was the first indication that smoking may be an important factor in this skin disease, possibly by affecting the inflammatory responses of the skin.