Pseudoxanthoma Elasticum and Elastosis Perforans Serpiginosa

This paper is intended to emphasize the significance of the association of elastosis perforans serpiginosa with heritable disorders of connective tissue. A case is reported in which a patient with pseudoxanthoma elasticum developed multiple lesions of elastosis perforans serpiginosa. A review of the literature revealed only two such cases previously reported. The cases of elastosis perforans serpiginosa previously reported in association with other diseases are summarized.

The case is that of a 35-year-old Caucasian male with the skin changes of pseudoxanthoma elasticum present since childhood. A sister also has this disease. Over the past seven years he developed multiple lesions of elastosis perforans serpiginosa which were located in the areas most affected by pseudoxanthoma elasticum. Seventeen such lesions were present at the time of examination. Histologic studies demonstrated the typical findings of both diseases.