Castillo CM, Moreno ME

Language: Spanish
References: 14
Page: 54-58
PDF size: 102.21 Kb.

ABSTRACT

The epidermal nevus syndrome is a very rare entity that has neurocutaneous lesions with systemic defects in the skin, brain, eyes and/or skeleton. The skin lesions of this syndrome are congenital hamar-tomas of ectodermal origin and they are classified on the basis of their main component and we can find four types. The nervous central system compromise include seizures, mental retardation and anatomyc alterations that involve cranial asymmetry, hemimegalencephaly with asymmetric and dilated ventricles and calcium deposit. We report a 24-years old female patient that has skin and systemic lesions compatibles with a Schimmelpenning-Feuerstein-Mims syndrome with CT and MRI studies those which showing the nervous central system compromise in this case.

REFERENCES

1. Robert A. Swartz. Epidermal nevus síndrome. www.eMedicine – March 9, 2005.

2. Su WP. Histopathologic varieties of epidermal nevus: a study of 160 cases. Am J Dermatol 1982;4:161-70.

3. Happle R. Epidermal nevus syndromes. Semin Dermatol 1995; 14:111-21.

4. Happle R. Gustav Schimmelpenning and the syndrome bearing his name. Dermatology 2004;209:84-7.

5. Happle R: How many epidermal nevus syndromes exist? A clinicogenetic classification. J Am Acad Dermatol 1991;25: 550-6

6. Bolognia JL, Orlow SJ, Glick ShA. Lines of Blaschko. J Am Acad Dermatol 1994; 31:157-90.

7. Vidaurri-de la Cruz H, Tamayo-Sanchez L, Duran-McKinster C, et al. Epidermal nevus síndromes: clinical findings in 35 patients. Pediatr Dermatol 2004;21:432-9.

8. Diven DC, Solomon AR, McNeely MC, et al. Nevus sebaceus associated with major ophthalmologic abnormalities. Arch Dermatol 1987;123:383-6.

9. Vles JS, Degraenwe P, De Cock P, Caser P. Neuroradiological findings in Jadassohn nevus phakomatosis: a report of four cases. Eur J Pediatr 1985;144:290-4.

10. Allison MA, Dunn CL, Pedarsen RC. What Síndrome is this? Epidermal nevus síndrome: a neurological variant with hemimegalencephaly, facial hemihypertrophy and giral malformtation. Pediatr Dermatol 1998;15:59-61.

11. Pavone L, Curatolo P, Rizzo R, et al. Epidermal nevus síndrome: a neurological variant with hemimegalencephaly, giral malformation, mental retardation, seizures and facial hemihypertrophy. Neurology 1991;4:266-71.

12. Sasaki M, Matsuda H, Arai Y, Hashimoto T. Startle-induced epilepsy in a patient with epidermal nevus Syndrome. Pediatr Neurol 1998;18:346-9.

13. Roger M, McCrosin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermat 1989;20:476-88.

14. Roger M, McCrosin I, Commens C: Epidermal nevi and the epidermal nevus syndrome. A review of 233 cases. Pediatr Dermatol 1992;9:342-4.