Incontinentia Pigmenti. Presented by Richard L. Kidd, MD, and George F. Wilgram, MD.
A 17-year-old girl had a peculiar pattern of pigmentation during the first year of life. During a recent hospitalization, vesicles developed on her legs with subsequent papuleformation; the dermatitis spontaneously subsided over two- to four-week periods. The patient's teeth, eyes, and skeletal, central nervous, and cardiac systems were normal.Physical examination revealed increased pigmentation widely distributed from the neck to the ankles, taking the appearance of splashed paint and marble. The dermatitis is most striking over the back.
Dr. Kidd: Dr. George F. Wilgram, Mr. Paul Cole, and I have had the opportunity to examine specimens of this girl's skin under the electron microscope. In addition to the pigmentary anomaly that we have reported elsewhere (to be published), the epidermis demonstrates a large number of Merkel cells in the basal layer. At least one Merkel cell
Fromer JL. NEW ENGLAND DERMATOLOGICAL SOCIETY. Arch Dermatol. 1972;105(5):767-774. doi:10.1001/archderm.1972.01620080085032