Dermatológia pre prax 1/2010
Morbus Bourneville-Pringle
This article reports a case of 55 old patient with histologically verified diagnosis of morbus Bourneville-Pringle (tuberous sclerosis), hereditary systemic neurocutaneous disease, belonging to phakomatoses. Beside of main clinical manifestations with papules on a face and paraungual Koenen’s tumors, authors describe diagnostic algorithm and treatment results – combination of antiprolipherative therapy (retinoids) and surgical extirpation as well.
Keywords: tuberous sclerosis, adenoma sebaceum, angiofibromas, epilepsy, mental retardation, surgical extirpation
