2.1.1. Dermal Manifestations

Skin lesions are generally the first signs of PXE to be observed during childhood or adolescence and often progress slowly and unpredictably. Therefore, a dermatologist frequently makes the initial diagnosis. The accumulation of abnormal calcified elastic fibers in the mid-dermis produces yellow-hued papules and plaques and laxity with loss of elasticity. These lesions can be seen on the neck, axilla, antecubital fossa, popliteal fossa, groin, and periumbilical area [25,30–32].
