Sahn E E, Garen P D, Pai G S, Levkoff A H, Hagerty R C, Maize J C
Department of Dermatology, Medical University of South Carolina, Charleston, SC 29425.
Am J Dermatopathol. 1990 Oct;12(5):485-91. doi: 10.1097/00000372-199010000-00009.
A case of multiple rhabdomyomatous mesenchymal hamartomas is presented. The patient is a black male infant, the product of an uncomplicated term gestation and delivery. At birth, there were numerous polyps distributed over the periorbital and periauricular areas bilaterally. Some appeared fingerlike with constrictions below their tips. Others were branched or globular in shape. These projections showed spontaneous and independent movement, particularly during feedings. On histopathologic examination, the polyps were covered by squamous epithelium and contained normal follicular units. Bundles of skeletal muscle were present in the reticular dermis, extending into the subcutis. Regular cross-striations were seen in these muscle fibers. In some specimens, the muscle bundles formed a solid, central core. Skeletal muscle histochemical stains confirmed the presence of both types 1 and 2 muscle fibers. Electron microscopy revealed a normal skeletal muscle banding pattern. This case is the first report of multiple rhabdomyomatous mesenchymal hamartomas of skin. Functional skeletal muscle with spontaneous movement is part of the clinical picture.
本文报告一例多发性横纹肌瘤样间叶性错构瘤。患者为一名黑人男婴,足月顺产,分娩过程顺利。出生时,双侧眶周和耳周区域有大量息肉。一些息肉呈指状,尖端下方有缩窄。其他息肉呈分支状或球状。这些突起表现出自发且独立的运动,尤其是在喂食期间。组织病理学检查显示,息肉被鳞状上皮覆盖,包含正常的毛囊单位。网状真皮层存在骨骼肌束,延伸至皮下组织。这些肌纤维可见规则的横纹。在一些标本中,肌束形成一个坚实的中央核心。骨骼肌组织化学染色证实存在1型和2型肌纤维。电子显微镜显示出正常的骨骼肌条纹模式。该病例是皮肤多发性横纹肌瘤样间叶性错构瘤的首例报告。具有自发运动的功能性骨骼肌是临床表现的一部分。