A 51-year-old white woman presented with a 17-year history of multiple, asymptomatic papules on her face, neck, and upper chest area. The papules had previously been diagnosed clinically as milia and later as angiofibromas for which she underwent a complete evaluation for tuberous sclerosis. A biopsy had not previously been performed on these lesions. The patient was otherwise healthy except that she had had a spontaneous pneumothorax the preceding year. Her sister, father, and paternal grandmother had also developed similar lesions during their 30s.

On physical examination, there were multiple 2- to 3-mm firm, dome-shaped, skin-colored to white papules scattered on the patient's face, neck (Figure 1), upper chest area, and axillae; she also had multiple skin-colored pedunculated papules on her neck. The nasolabial folds and upper vermilion border were spared. Four skin biopsy specimens were obtained, all of which showed similar findings (Figure 2 and Figure 3).