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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
Full text How to cite this article

Language: Spanish
References: 9
Page: 314-317
PDF size: 433.25 Kb.


Key words:

tuberous sclerosis, periungual fibromas, Köenen tumors.

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

  1. Palacios López CG, Complejo esclerosis tuberosa en pac Dermatología3, Programa de Actualización Continua en Dermatología, 1ª ed., México,Intersistemas, pp. 199-205.

  2. Tsao H y Luo S, Neurofibromatosis and tuberous sclerosis. En BologniaJL, Jorizzo J y Schaffer JV, Dermatology, Elsevier, 2012, p. 933.

  3. Monteiro T, Garrido C, Pina S y Chorão R, Esclerosis tuberosa: caracterizaciónclínica e intento de correlación fenotipo/genotipo, AnPediatr 2014; 81(5):289-96.

  4. Castaño MR, Rosado RR y Bueno MR, Tumor de Köenen: etiología,diagnóstico diferencial y tratamiento de elección, 2015.

  5. Gómez Cerdas MT y Vargas Morales JA, Esclerosis tuberosa, Rev MédSinerg 2019; 4(3):21-37.

  6. Ma D, Darling T, Moss J y Lee CC, Histologic variants of periungualfibromas in tuberous sclerosis complex, J Am Acad Dermatol 2011;64(2):442-4.

  7. Longhurst WD y Khachemoune A, An unknown mass: the differentialdiagnosis of digit tumors, Int J Dermatol 2015; 54(11):1214-25.

  8. 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.

  9. 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.




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C?MO CITAR (Vancouver)

Dermatología Cosmética, Médica y Quirúrgica. 2022;20