Papulosis fibroelastolítica del cuello. ¿Es la elastólisis de la dermis papilar un hallazgo histopatológico característico?

Cobblestone plaques on the lateral side of the neck

“…In 1997, Balus et al 1 coined the term of fibroelastolytic papulosis of the neck (FEPN) to encompass similar clinicopathological entities such as pseudoxanthoma elasticum with papillary dermal elastolysis (PXE‐PDE) and white fibrous neck papulosis (WPFN) 1,2 . FEPN is a rare disease, with acquired idiopathic, noninflammatory lesions, reported mainly in the neck or supraclavicular region and from the fifth decade of life onwards 2,3 . The pathogenesis of FEPN is unknown, although it is thought to be related to skin aging, ultraviolet radiation, and elastogenesis 3 .…”

“…FEPN is a rare disease, with acquired idiopathic, noninflammatory lesions, reported mainly in the neck or supraclavicular region and from the fifth decade of life onwards 2,3 . The pathogenesis of FEPN is unknown, although it is thought to be related to skin aging, ultraviolet radiation, and elastogenesis 3 . Clinically, FEPN presents with yellowish or whitish, asymptomatic or mildly pruritic papules that may coalesce into plaques in a “cobblestone” pattern somewhat resembling PXE 4 .…”

“…The histological diagnosis is characterized by a marked decrease in the elastic fibers of the upper and middle dermis visualized with special stainings for elastic tissue, usually accompanied by sclerosis of the collagen fibers 2 . Some authors suggest that the presence of papillary dermal elastolysis in both affected and unaffected areas would suggest that this finding should not be considered a characteristic diagnostic feature 3 . Unlike PXE, in FEPN there is an absence of mineralization on histology as staining for calcium is negative, and no associated systemic manifestations have been reported 4,5 .…”