Pseudoxanthoma elasticum is a rare congenital genetic elastolytic disorder that is characterized by multiple and coalescing soft, yellowish papules that are symmetrically distributed on the neck and other flexural body areas. Associated findings include cardiac and ophthalmologic alterations. In our cases, dermoscopy revealed the presence of multiple irregular, yellowish areas alternating with prominent linear vessels. The yellowish hue may be related both to the elastolysis of elastic fibers or the presence of calcium deposits. The prominent superficial linear vessels may be caused by a vascular rearrangement driven by the underlying dermal elastolysis. This peculiar aspect has not been previously described and, if confirmed by studies on several patients, could be useful for a noninvasive prompt diagnosis of pseudoxanthoma elasticum in order to appropriately address diagnostic work-up and follow-up.
Dermoscopy of pseudoxanthoma elasticum.
Lacarrubba F;Verzì AE;CALTABIANO, ROSARIOPenultimo
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2017-01-01
Abstract
Pseudoxanthoma elasticum is a rare congenital genetic elastolytic disorder that is characterized by multiple and coalescing soft, yellowish papules that are symmetrically distributed on the neck and other flexural body areas. Associated findings include cardiac and ophthalmologic alterations. In our cases, dermoscopy revealed the presence of multiple irregular, yellowish areas alternating with prominent linear vessels. The yellowish hue may be related both to the elastolysis of elastic fibers or the presence of calcium deposits. The prominent superficial linear vessels may be caused by a vascular rearrangement driven by the underlying dermal elastolysis. This peculiar aspect has not been previously described and, if confirmed by studies on several patients, could be useful for a noninvasive prompt diagnosis of pseudoxanthoma elasticum in order to appropriately address diagnostic work-up and follow-up.File | Dimensione | Formato | |
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