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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

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