Sidhu G S, Cassai N D, Rico M J
New York Harbor Healthcare System (NYHHS) and New York University School of Medicine, New York, USA.
Ultrastruct Pathol. 2000 Nov-Dec;24(6):391-7. doi: 10.1080/019131200750060050.
The gross, light microscopic, and ultrastructural findings in a 55 year old man was striate keratoderma are presented. There was no family history of the disease. The lesions developed in his late teens and early adult years, and consisted of progressively worsening, raised, hyperkeratotic, linear plaques on the palm and volar surface of the third and fifth fingers bilaterally. There were also painful callosities on both heels, and thick, raised plaques on the heels and lateral plantar surfaces. The epidermis was papillomatous and acanthotic, with marked orthokeratosis, minimal parakeratosis, and a very thickened granular layer. No epidermolysis was seen. Electron microscopy showed increased tonofibrils in the stratum spinosum arranged in wavy, parallel bundles and a granular layer in which normal Odland bodies were present. However, the keratohyaline granules were large, with rounded borders and a striped, alternating, dark and light content characteristic of composite granules. There was diminished contact of the granules with tonofibrils. The transition to the stratum corneum was abrupt. The ultrastructural and genetic features of keratodermas, with special emphasis on the striate type, are reviewed.
本文报告了一名55岁患有条纹状角化病男性的大体、光镜和超微结构检查结果。该疾病无家族病史。病变在他青少年晚期和成年早期出现，表现为双侧手掌以及第三和第五指掌面逐渐加重的、隆起的、角化过度的线性斑块。双侧足跟也有疼痛性胼胝，足跟和足底外侧表面有增厚、隆起的斑块。表皮呈乳头瘤样且棘层肥厚，有明显的正角化，轻度不全角化，颗粒层非常增厚。未见表皮松解。电子显微镜检查显示棘层中张力原纤维增加，呈波浪状平行束状排列，颗粒层中有正常的奥尔德小体。然而，透明角质颗粒较大，边界圆润，具有复合颗粒特有的条纹状、交替的明暗内容物。颗粒与张力原纤维的接触减少。向角质层的过渡很突然。本文综述了角化病的超微结构和遗传学特征，特别强调了条纹状类型。
