Morita K, Hogan M E, Nanney L B, King L E, Manabe M, Sun T T, Sundberg J P
Department of Medicine, Vanderbilt School of Medicine, Nashville, TN, USA.
J Dermatol. 1995 Jun;22(6):385-95. doi: 10.1111/j.1346-8138.1995.tb03412.x.
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.
一种常染色体隐性遗传病，其临床和组织病理学皮肤特征类似于人类寻常型银屑病，自然发生于片状皮肤小鼠（fsn/fsn）。患病小鼠出生时除了低色素性贫血外一切正常。随后，它们会出现角化过度斑块和棘皮症，并伴有 rete 嵴延长。扫描电子显微镜检查显示表皮显著增厚，毛发稀疏，表皮表面有鳞屑堆积。毛干有明显的凹坑、条纹和外生性突起。指甲弯曲成 90 度角，表面不规则，指甲根部有鳞屑堆积。透射电子显微镜检查显示表皮厚度增加、线粒体异常以及中性粒细胞侵入表皮内。使用丝聚合蛋白原免疫细胞化学染色检测到透明角质颗粒异常。在真皮 - 表皮交界处，可见大量巨噬细胞和肥大细胞紧邻基底膜的局灶性溶解。乳头层真皮中可见高密度的胶原纤维和细胞浸润。这种超微结构异常的组合通常见于寻常型银屑病，并支持片状皮肤小鼠突变是人类寻常型银屑病一种自然发生的类似物这一理论。
