Abstract
Routine elastic tissue stains reveal an increased concentration of dermal elastic tissue. The fully developed elastic fiber with abundant elastin is the primary target in PXE. The oxytalin and elaunin fibers in that papillary dermis, which are composed predominantly of microfibrils, are spared. The von Kossa stain for carbonate or phosphate is the best and most sensitive stain to detect early (or late) calcification. Early changes are not visible with H&E staining. In more advanced lesions, the foci of fully calcified and fragmented fibers take on a bluish hue with H&E staining methods. Advanced lesions show positive von Kossa staining around eccrine sweat glands and in the elastic lamina of larger cutaneous vessels. Alizarin red stains for calcium are positive and identify the cation as calcium. Alcian blue stains are positive for acid mucopolysaccharides, showing pooling throughout the dermis. Interpretation is often difficult. Periodic acid-Schiff stains show no abnormalities. Collagen appears normal on all stains. Arteries from surgical specimens may show calcification in the elastic laminae, confirming the systemic nature of PXE.
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