Papillary Dermal Elastolysis with a Twist

PXE-like PDE is likely underdiagnosed rather than rare, and dermatologists should know how to differentiate it from inherited PXE.

Pseudoxanthoma elasticum–like papillary dermal elastolysis (PXE-like PDE) is a rare, acquired, complete absence of oxytalan and elaunin elastic fibers in the papillary dermis. Not only is fibrillin-1 absent in these patients (as in solar-damaged skin), but also elastin. Therefore, the etiology of PXE-like PDE is thought to be multifactorial, involving intrinsic and extrinsic factors.

In this prospective study, researchers analyzed clinical and pathologic findings in 17 women (mean age, 61.8 years) with PXE-like PDE. The clinical lesions were asymptomatic, dome-shaped, nonfollicular, soft papules that joined to form yellow “cobblestone” plaques. Skin biopsy specimens were subject to hematoxylin-eosin, Weigert, Verhoeff-van Gieson, or orcein s…

Reviewing Author

Angelica Selim, MD

Disclosures

Grant / Research support NIH/NCI, The Plastic Surgery Foundation

Angelica Selim, MD

Disclosures

Grant / Research support NIH/NCI, The Plastic Surgery Foundation