Periumbilical perforating pseudoxanthoma elasticum ‐ an acquired perforating disorder

Two cases of periumbilical perforating pseudoxanthoma elasticum with dermoscopic and histopathologic features

“…In a discussion of a rare acquired dermatosis, a debate has ensued since the original description of periumbilical perforating pseudoxanthoma elasticum (PXE) as to whether it is a separate entity or a localised purely cutaneous variant . Another viewpoint suggests that it is a bridge between the inherited and acquired forms of PXE …”

“…The purported pathogenesis is stretching of the abdominal wall connective tissue, for example, in multiparous and obese patients causing alteration and calcification of elastic fibres which undergo transepidermal elimination. It classically presents as a periumbilical hyperpigmented plaque on which keratotic ulcerated papules develop. Dermoscopy gives a new perspective as discussed below.…”

“…Periumbilical perforating pseudoxanthoma elasticum is a rare acquired disorder, whose classification is still debatable . It may be a localised cutaneous variant type of pseudoxanthoma elasticum (although it may affect internal organs) or a separate disease entity . It classically presents with mildly pruritic keratotic papules on a periumbilical yellowish plaque.…”

“…Few reports have been published detailing the dermoscopic features of this disease. This disease may appear placid; however, systemic comorbidities (especially vascular) are reported in a proportion of patients . We present two such cases with dermoscopic and histopathologic features.…”

Pseudoxanthoma elasticum: the paradigm of ectopic mineralization disorders – diagnosis and treatment

“…An unusual presentation of PXE is the perforating variant, often encountered in obese multiparous women of African ancestry in periumbilical distribution [13]. These cutaneous findings are occasionally associated with angioid streaks.…”