The term FEP encompasses pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). These are 2 clinically and histopathologically similar conditions that are believed by some to be variants of the same disease spectrum.
FEP has been reported in both men and women, typically those who are between 40 and 80 years old. It presents as asymptomatic or mildly pruritic whitish-yellow, soft papules that tend to coalesce into cobblestone-patterned plaques, usually on the neck. Sometimes, the papules may be more white, discrete, and firm. More rarely affected areas include the supraclavicular region, upper torso, and the flexural and intertriginous areas.
The pathophysiology of FEP is not fully understood, although it has been hypothesized that intrinsic aging plays a key role in its development. The disorder tends to be slowly progressive with time.
L98.8 – Other specified disorders of the skin and subcutaneous tissue
402681005 – Disorder due to abnormality of dermal elastin
Differential Diagnosis & Pitfalls
- Pseudoxanthoma elasticum – Papules typically develop in young adults. Often has systemic involvement (gastrointestinal, cardiovascular, ocular). Differentiate with skin biopsy.
- Mid-dermal elastolysis – Predominantly affects trunk and back.
- Linear focal elastosis – Palpable, yellowish striae on mid-lower back.
- Solar elastosis