Possible explanations for elastolysis include defective elastin synthesis, uncontrolled release of elastase by inflammatory cells, elastophagocytosis, or an immune mechanism. Familial anetoderma is uncommon with only 7 families reported in the literature. Inheritance may be autosomal dominant, autosomal recessive, or undefined in pattern. This suggests that Borrelia may be involved. Initial primary lesions may be noticed as crops of asymptomatic erythematous macules, plaques, nodules, or urticarial wheals that enlarge over weeks to reach several centimeters in size.
Lesions appear on the upper arms, trunk, and thighs.
Comorbid lesions may already exist. End-stage lesions do not change over time, and new lesions may continue to develop over years. On pressure, a normal ring of surrounding skin is felt. The scalp, palms, soles, and mucous membranes usually are spared. Very few to hundreds of lesions may form.