title: Cutaneous ultrastructural features of the flaky skin (fsn) mouse mutation.
abstract: An autosomal recessive genetic disease with clinical and histopathological skin features resembling human psoriasis vulgaris occurs naturally in flaky skin mice (fsn/fsn). Affected mice are normal at birth, except for a hypochromic anemia. Subsequently, they develop hyperkeratotic plaques and acanthosis with elongation of rete ridges. Scanning electron microscopic examination revealed a greatly thickened epidermis, a sparsity of hairs and scale accumulations on the epidermal surface. Hair shafts had conspicuous pits, striations, and exophytic protrusions. Nails were bent at a 90 degrees angle with surface irregularities and accumulations of scale at the nail base. Transmission electron microscopic examination showed increased epidermal thickness, mitochondrial aberrations, and intraepidermal invasion by neutrophils. Keratohyalin abnormalities were detected using immunocytochemical staining for profilaggrin. At the dermal-epidermal junction, numerous macrophages and mast cells were seen in close proximity to focal dissolutions of the basement membrane. A high density of collagen fibers and cellular infiltrates were evident in the papillary dermis. This constellation of ultrastructural aberrations is typically found in psoriasis vulgaris and supports the theory that the flaky skin mouse mutation is a naturally occurring analog to one variety of human psoriasis vulgaris.
authors.affiliation: Department of Medicine, Vanderbilt School of Medicine, Nashville, TN, USA.
authors.forename: K
authors.initials: K
authors.lastname: Morita
authors.forename: M E
authors.initials: ME
authors.lastname: Hogan
authors.forename: L B
authors.initials: LB
authors.lastname: Nanney
authors.forename: L E
authors.initials: LE
authors.lastname: King
authors.forename: M
authors.initials: M
authors.lastname: Manabe
authors.forename: T T
authors.initials: TT
authors.lastname: Sun
authors.forename: J P
authors.initials: JP
authors.lastname: Sundberg
mesh_terms: D000747:Anemia, Hypochromic / Q000235:genetics; D000818:Animals; D001485:Basement Membrane / Q000648:ultrastructure; D004195:Disease Models, Animal; D004817:Epidermis / Q000648:ultrastructure; D000091344:Filaggrin Proteins; D005808:Genes, Recessive; D006197:Hair / Q000002:abnormalities / Q000648:ultrastructure; D006801:Humans; D007381:Intermediate Filament Proteins / Q000032:analysis; D007642:Keratosis / Q000473:pathology; D051379:Mice; D008817:Mice, Mutant Strains / Q000033:anatomy & histology* / Q000235:genetics; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning; D009262:Nails / Q000648:ultrastructure; D009264:Nails, Malformed; D010750:Phosphoproteins / Q000032:analysis; D011498:Protein Precursors / Q000032:analysis; D011565:Psoriasis / Q000235:genetics / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure*
chemical_list: D000091344:Filaggrin Proteins; D007381:Intermediate Filament Proteins; D010750:Phosphoproteins; D011498:Protein Precursors