Systemic elastorrhexis is a widespread hereditary degeneration of mesodermal tissues affecting many structures in the body, particularly the dermis, the media of the arteries, and Bruch's membrane in the eye. Pseudoxanthoma elasticum is one form of elastorrhexis mainly affecting the skin. Degeneration of the elastic lamina of Bruch's membrane results in the formation of angioid streaks in the r...

Systemic elastorrhexis is a widespread hereditary degeneration of mesodermal tissues affecting many structures in the body, particularly the dermis, the media of the arteries, and Bruch's membrane in the eye. Pseudoxanthoma elasticum is one form of elastorrhexis mainly affecting the skin. Degeneration of the elastic lamina of Bruch's membrane results in the formation of angioid streaks in the r...

The babysitter-procedure might offer an alternative when nerve reconstruction is delayed in order to overcome muscular atrophy due to denervation. In this study we aimed to show that a sensomotoric babysitter-procedure after median nerve injury is capable of preserving irreversible muscular atrophy. The median nerve of 20 female Wistar rats was denervated. 10 animals received a sensory protecti...

BACKGROUND Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. METH...

THE PHYSICAL APPEARANCE of aged skin derives from a combination of intrinsic and extrinsic factors. Intrinsic changes are largely genetically determined and include the effects of gravity (sagging), expression lines, and atrophy of the dermis and subcutaneous tissue. Extrinsic aging is related to environmental influences with sunlight being the most important, followed by smokingandchemicalexpo...

INTRODUCTION Biotinidase deficiency (BTD) is an inborn error of biotin metabolism inherited as an autosomal recessive trait. Due to the, biotinidase deficiency, biotin is not recycled. Individuals with BTD usually exhibit neurological and cutaneous abnormalities unless treated with biotin. Supplementation with biotin may either ameliorate or if early introduced even prevent symptoms when introd...

BACKGROUND Onchocerciasis results from infestation by the nematode Onchocerca volvulus, and is characterized clinically by troublesome itching, skin lesions and eye manifestations. Since 1992, approximately 9,000 immigrants have arrived in Israel from the Kuwara province of northwest Ethiopia where the prevalence of onchocerciasis is particularly high. OBJECTIVES To determine whether onchocer...

Sir, Atopic dermatitis (AD) is associated with genetic and environmental factors, defects in the cutaneous barrier, bacterial and viral skin infections and immunological changes (1). AD influences the quality of life of both the patient and his or her family (2). Topical steroids are the gold standard treatment for AD, but their use is limited by potential adverse effects, including impairment ...

A 16-month-old male presented with focal motor seizures with secondary generalization. On physical exam, a bilateral facial cutaneous angioma, sharply demarcated by the distribution of the ophthalmic and maxillary branches of the trigeminal nerve, was noted (Figure 1). Magnetic resonance imaging (MRI) demonstrated calcification and atrophy of the right frontal lobe as well as an associated pial...