The neurofibromatoses are members of the group of neurocutaneous disorders which also include tuberous sclerosis, neurocutaneous melanosis, hypomelanosis of Ito, incontinentia pigmenti, Sturge-Weber syndrome and von Hippel-Lindau disease. Collectively, these conditions have also been referred to as "phakomatoses," a term is derived from the Greek phakos, meaning a lentil or birthmark. All but S...

We present a 12-month-old Hispanic female with a reticulated, hyperchromic rash in a striated pattern appearing on upper and lower extremities and trunk and back since the age of 6 weeks. Over the next 10 months, the rash persisted. The rash did not respond to treatment with antifungals and steroids. During her 6-month wellness visit, the patient was diagnosed with incontinentia pigmenti (IP), ...

Incontinentia pigmenti (IP) is an X-linked dominant genodermatosis characterized by typical skin lesions along Blaschko's lines and associated with ocular, dental, nails, hair, skeletal, central nervous system and cardiovascular anomalies. We report a 5-year-old boy with cutaneous hyperpigmentation along Blaschko's lines, atrophic streaks, strabismus and mental retardation. He showed the charac...