PubMed · 9550319
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R S Simón, E Sánches Yus. 1998. Does eccrine hidrocystoma exist?. https://doi.org/10.1111/j.1600-0560.1998.tb01713.x
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Eccrine angiomatous hamartoma is a rare condition characterized histologically by increased numbers of eccrine structures and numerous capillary channels. Patients characteristically have a solitary, congenital nodule that may be painful and that may show hyperhidrosis. It is important to recognize this condition because it is a benign lesion for which aggressive treatment is not indicated. We report the case of a congenital eccrine angiomatous hamartoma that had a firm nodule studded with blue papules.
The histologic diagnosis as eccrine hidrocystoma may contain some other cysts which do not originate from the eccrine sweat glands. Some authors have suggested that true eccrine hidrocystoma does not exist. However, multiple eccrine hidrocystoma (Robinson type) is a distinct clinical entity which is characterized by multiple translucent papules around the eyelids which appear in warm weather. Cytokeratin expression in 8 cases of multiple type of unequivocal eccrine hidrocystoma was analyzed immunohistochemically. Immunoreactivities in our cases were similar to those of eccrine dermal ducts. Therefore, we confirmed that these tumors were derived from the eccrine dermal duct, not from the other appendages, and that true eccrine hidrocystoma exists.
As an investigation of the pathogenetic mechanism of diminished sweating in Fabry disease, an electron microscopy ultrastructural study was conducted on specimens of eccrine sweat glands from a typical patient with Fabry disease who had hypohidrosis, a low skin moisture content, and diminished thermoregulation ability. Numerous characteristic cytoplasmic inclusions were observed in the eccrine sweat glands, the lamellar pattern of which was considerably variable in various types of gland cells. Large vacuolar inclusions predominated in clear cells of secretory coil; lesser vacuoles were also seen in the coiled duct, and the basal cells of the straight duct toward the coiled duct displayed mulberry-like figures. There were some clear cells showing cell damage and necrosis in the secretory coil. Lamellated inclusions were noted in the unmyelinated axons innervating the eccrine sweat glands. The small blood vessels around the eccrine glands were narrowed by swollen endothelial cells with heavy inclusions. These intracytoplasmic deposits may be responsible for the decreased sweating ability in Fabry disease. The factors related to hypohidrosis are also discussed.