Kaposiform hemangioendothelioma

There are rare but important causes of neonatal blistering, diaper rash, and solitary skin nodules. Incontinentia pigmenti (IP), for example, is an X-linked dominant disorder affecting the skin, eyes, and central nervous system. IP is suspected when blisters occur in a blaschkoid distribution. Because IP is lethal in males, presentation of a female patient whose mother has a history of miscarriages can indicate this disease. IP can be diagnosed based solely on rash and skin biopsy results, without genetic testing. Early diagnosis and treatment can significantly reduce morbidity and mortality by preventing seizures and promoting development.