The mucocutaneous marker of pseudoxanthoma elasticum

A patient with angioid streaks discovered ophthalmoscopically was evaluated in the dermatology clinic for pseudoxanthoma elasticum (PXE). Cutaneous markers for PXE did not appear in the usual locations; mucosal involvement of the lower lip was the only clinically ascertainable clue. The presence of the disease was confirmed by histologic examination of the oral mucosa. This case stresses the importance of familiarity with the mucocutaneous markers of PXE by members of the dental profession.