2022, Number 3
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Dermatología Cosmética, Médica y Quirúrgica 2022; 20 (3)
Köenen tumor: a rare cutaneous manifestation of tuberous sclerosis
Dávalos TM, López MRK, Palacios LC
Language: Spanish
References: 9
Page: 314-317
PDF size: 433.25 Kb.
ABSTRACT
Tuberous sclerosis is an autosomal dominant neurocutaneous
syndrome with an incidence of 1 in 10 000 births. The classic
triad of the disease are seizures, hypomelanotic macules and
angiofibromas. The cutaneous manifestations are considered
markers of the disease due to their frequency and considerable
number in one patient.
Periungual or subungual fibromas, also known as Köenen
tumors, are considered a major criterion of the disease, however
they are not present at the time of diagnosis. They appear
during adolescence and they are a frequent cause of consultation
since they produce pain and inability to walk in these patients.
We present a case of a child with tuberous sclerosis and
a Köenen tumor, treated surgically with good outcome.
REFERENCES
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Castaño MR, Rosado RR y Bueno MR, Tumor de Köenen: etiología,diagnóstico diferencial y tratamiento de elección, 2015.
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Longhurst WD y Khachemoune A, An unknown mass: the differentialdiagnosis of digit tumors, Int J Dermatol 2015; 54(11):1214-25.
Liebman JJ, Nigro LC y Matthews MS, Köenen tumors in tuberoussclerosis: a review and clinical considerations for treatment, Ann PlastSurg 2014; 73(6):721-2.
Martínez Campayo N, Taibo Martínez A, Del Pozo Losada J y FonsecaE, Shave and carbon dioxide laser vaporization of ungual fibromas,Koenen’s tumors, in five patients with tuberous sclerosis, Dermatol Ther2021; 34(1):e14571.