Pringle-bourneville phacomatosisQ85.1
HistoryThis section has been translated automatically.
Bourneville, 1880; Pringle, 1890
DefinitionThis section has been translated automatically.
Full picture of the neurocutaneous malformation syndrome described by Bourneville in 1880 (old term: phacomatosis) with:
- adenoma sebaceum
- lumbosacral connective tissue nevus
- Ash-leaf stains
- Koenen tumour
- tuberous cerebral sclerosis (see below tuberous sclerosis)
- Angioleiomyomas (see below lipoma)
- mental retardation and seizure disorder.
S. and Tuberous Sclerosis
Occurrence/EpidemiologyThis section has been translated automatically.
Prevalence of 1:10,000
TherapyThis section has been translated automatically.
Not known. Symptomatic treatment by clinic, see there.
LiteratureThis section has been translated automatically.
- Borelli S (1999) Koenen, Kothe and peri-ungual fibroma in tuberous sclerosis. dermatologist 50: 368-369
- Bourneville DM (1880) Sclérose tubéreuse des circonvolution cérébrales: Idioty and epilepsy hemiplégique. Arch Neurol (Paris) 1: 81-9l
- Bourneville DM, Brissaud E (1881) Encéphalite ou sclérose tubéreuse des circonvolutions cérébrales. Arch Neurol (Paris) 1: 390-412
- Burrows NJ et al (2004) Pulmonary artery aneurysm and tuberous sclerosis. Thorax 59: 86
- Narayanan V (2003) Tuberous sclerosis complex: genetics to pathogenesis. Pediatric Neurol 29: 404-409
- Pringle JJ (1890) A case of congenital adenoma sebaceum. Br J Dermatol 2: 1-14
- Pringle JJ (1890) On a case of congenital adenoma sebaceum. Monatsh prakt Dermatol (Leipzig) 10: 197-211
- Rieger E et al (1992) The clinical spectrum of orangan manifestations in tuberous sclerosis. Dermatologist 43: 272-277