Clinical applications of magnesium in cutaneous medicine
Kupetsky A. Erine in Magnesium, 2019
This chapter discusses potential applications of magnesium as adjunctive therapy in cutaneous conditions including pseudoxanthoma elasticum, psoriasis, hidradenitis suppurativa, cutaneous lupus erythematosus, mycosis fungoides, melanoma, acne vulgaris, melasma, Hailey–Hailey disease, and atopic dermatitis. Magnesium is a nutritionally essential element that is necessary for many physiologic processes in humans. It is a critical factor in bone mineralization, muscle contraction, nerve impulse transmission, and a necessary co-factor for over 300 enzymes. Magnesium has several dosage formulations pertaining to cutaneous treatment, including oral supplementation and topical variants. The dietary allowance of magnesium recommended by the Food and Drug Administration is up to 420 mg per day for adults. Fumaric acid esters (FAE) are another prospective oral supplementation of magnesium. Fumaderm is the only licensed FAE in Germany; it is comprised of dimethylfumarate and calcium, magnesium, and zinc salts of monoethyl hydrogen fumarate tablets.
Ophthalmology
Fazal-I-Akbar Danish in Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Angioid streaks: 1 Pituitary disorder (acromegaly etc). 2 Bone disorder (Paget’s disease). 3 Connective tissue disorder (Ehlers–Danlos’ syndrome; pseudoxanthoma elasticum). 4 Blood disorder (sickle-cell disease).
Pseudoxanthoma Elasticum
Charles Theisler in Adjuvant Medical Care, 2022
Pseudoxanthoma elasticum (PXE), also known as Grönblad-Strandberg syndrome, causes yellow papules, or bumps, to develop on the neck, underarms, and other areas of skin friction. It is a progressive genetic disease in which calcium deposits and other minerals accumulate in elastic fibers. Mineralization of elastic fibers primarily affects the skin, the retina, and blood vessels. On occasion, the digestive tract can be also be involved. Complications include eye abnormalities which can involve bleeding and scarring of the retina, leading to vision loss. Atherosclerosis and claudication are other frequent complications.
Approach to the management of pregnancy in patients with pseudoxanthoma elasticum: a review
Published in Journal of Obstetrics and Gynaecology, 2016
Marta Camacho, Cristobal Rengel, Elisa López-Herrero, Juan L. Carrillo, Agustín J. Eslava, Pedro Valdivielso
Management of pregnancy in patients with rare diseases is often guided by incomplete knowledge because of a lack of high-quality case control studies or single-centre experience. Pseudoxanthoma elasticum (PXE) is an autosomal recessive metabolic disorder that results in calcification of elastic fibres of the skin, retina, and arteries, leading to skin lesions, eventual central visual loss, and potential arterial insufficiency in most patients. It is due to mutations in ABCC6, which encodes the eponymous membrane transport protein. We review the literature on pregnancy in PXE, including the effects of the diseases on pregnancy and its complications, the effect of PXE on the foetus, and the effects of pregnancy on PXE, and conclude that in the majority of pregnancies in women with PXE, the outcome for mother, baby, and the disease is uneventful. We also provide recommendations for managing pregnancy in PXE.
Pseudoxanthoma Elasticum-Like in β-Thalassemia Major, a matter of α-Klotho and Parathyroid Hormone?
Published in Hemoglobin, 2017
Alessandro Baldan, Andrea Giusti, Cristina Bosi, Cristina Malaventura, Gianluca Forni, Caterina Borgna-Pignatti
Pseudoxanthoma elasticum-like (PXL) condition is one of the complications faced by patients with β-thalassemia major (β-TM). Histopathological features include abnormal, mineralized and fragmented elastic fibers in skin, eyes and arterial blood vessels (elastorrhexia). The pathogenesis of PXL lesions in β-TM is not yet completely understood. This study was aimed at analyzing a possible implication of α-Klotho in the clinical manifestation of PXL in patients with β-TM (30 with and 78 without PXL). A significant correlation was observed between Klotho, parathyroid hormone (PTH) and serum calcium (Ca). Our analysis seems to indicate α-Klotho and PTH as factors that can affect the development of PXL.
Is classical pseudoxanthoma elasticum a consequence of hepatic ‘intoxication’ due to ABCC6 substrate accumulation in the liver?
Published in Expert Review of Endocrinology & Metabolism, 2013
Mie Rostved Rasmussen, Mette Sommerlund, Søren Kragh Moestrup
Pseudoxanthoma elasticum (PXE) is a serious genetic disorder with ectopic mineralization affecting the skin, the eye and the cardiovascular system. The disease is predominantly caused by mutations in the transmembrane ABC protein ABCC6, a putative small substrate transporter. Interestingly, ABCC6 seems virtually absent in the affected organs, whereas a high expression is seen in hepatocytes. This and further published experimental evidence indicate that PXE is a systemic, metabolic liver disease where circulatory changes affect the peripheral mineralization process. Owing to the well-characterized transport of organic substrates by related ABC proteins, it has been proposed that PXE is caused by impaired export of an antimineralization compound to the blood. The authors here present an alternative hypothesis that explains ectopic mineralization in PXE as a consequence of hepatic accumulation of ABCC6 substrate(s) that via gene-regulating effects leads to altered hepatic secretion and activation of antimineralization/anticalcification proteins such as fetuin-A and Gla proteins.
Related Knowledge Centers
- Blood Coagulation
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- Genetic Skin Diseases
- Skin Abnormalities