G Kolde1, R Bachus, A C Ludolph. 1. Department of Dermatology, Virchow-Clinics, Humboldt-University of Berlin, Germany.
Abstract
BACKGROUND: Patients with sporadic amyotrophic lateral sclerosis (ALS) show disorganised collagen and elastin of the dermis. We looked for inflammatory alterations to cutaneous blood vessels. PATIENTS AND FINDINGS: Seven patients with sporadic ALS were investigated; five were confined to bed, but none had bedsores. Light and electron microscopy of skin showed an oedematous dermis with collagen fibrils of irregular diameter. Small blood vessels were characterised by duplicated basement membranes and deposition of beta-amyloid protein, the main component of the neuronal and non-neuronal amyloid deposits in Alzheimer's disease. These skin changes were seen in all degrees of disability, but none was found in age-matched and sex-matched controls. INTERPRETATION: The skin in ALS is characterised by a distinctive pattern of alterations of connective tissue and blood vessels. Examination of skin in an additional and easily accessible investigation which may help elucidate the pathogenesis of ALS.
BACKGROUND: Patients with sporadic amyotrophic lateral sclerosis (ALS) show disorganised collagen and elastin of the dermis. We looked for inflammatory alterations to cutaneous blood vessels. PATIENTS AND FINDINGS: Seven patients with sporadic ALS were investigated; five were confined to bed, but none had bedsores. Light and electron microscopy of skin showed an oedematous dermis with collagen fibrils of irregular diameter. Small blood vessels were characterised by duplicated basement membranes and deposition of beta-amyloid protein, the main component of the neuronal and non-neuronal amyloid deposits in Alzheimer's disease. These skin changes were seen in all degrees of disability, but none was found in age-matched and sex-matched controls. INTERPRETATION: The skin in ALS is characterised by a distinctive pattern of alterations of connective tissue and blood vessels. Examination of skin in an additional and easily accessible investigation which may help elucidate the pathogenesis of ALS.
Authors: Anika M Helferich; Sarah J Brockmann; Jörg Reinders; Dhruva Deshpande; Karlheinz Holzmann; David Brenner; Peter M Andersen; Susanne Petri; Dietmar R Thal; Jens Michaelis; Markus Otto; Steffen Just; Albert C Ludolph; Karin M Danzer; Axel Freischmidt; Jochen H Weishaupt Journal: Cell Mol Life Sci Date: 2018-07-20 Impact factor: 9.261
Authors: Alice Brockington; Ke Ning; Paul R Heath; Elizabeth Wood; Janine Kirby; Nicolò Fusi; Neil Lawrence; Stephen B Wharton; Paul G Ince; Pamela J Shaw Journal: Acta Neuropathol Date: 2012-11-13 Impact factor: 17.088