Abstract
The report focuses on a rare variant form of epidermal nevus syndrome (ENS) (Schimmelpenning-Feuerstein-Mims syndrome) describing lesions involving the skin, eyes, skeleton, heart and brain in an 11-year-old boy. Despite his evident brain pathology, the boy lacks neurological symptoms and mental retardation. We describe his unusual MRI appearances and radiographic skeletal findings. To our knowledge this is the first report of ENS with lymphoedema occurring together in the same individual.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lever WF, Schaumburg-Lever G (1983) Tumors of the dermal appendages. In: Lever WF, Schaumburg-Lever G (eds) Histopathology of the skin, 6th edn. Lippincott, Philadelphia, pp 578–650
Jones EW, Heyl T (1970) Naevus sebaceous. A report of 140 cases with special regard to the development of malignant tumours. Br J Dermatol 82:99–103
Happle R (1995) Epidermal nevus syndromes. Semin Dermatol 14:111–121
Schimmelpenning GW (1983) Langjährige verlaufskontrolle einer organoiden nävusphakomatose (Schimmelpenning-Feuerstein-Mims). Fortschr Röntgenstr 139:63–67
Sato K, Kubota T, Kitai R (1994) Linear sebaceous nevus syndrome (sebaceous nevus of Jadassohn) associated with abnormal neuronal migration and optic glioma: case report. Neurosurgery 35:318–320
Gurecki P, Holden KR, Sahn EE, et al (1996) Developmental neural abnormalities and seizures in epidermal nevus syndrome. Dev Med Child Neurol 38:716–723
Vles JS, Degraeuwe P, DeCock P, et al (1985) Neuroradiological findings in Jadassohn nevus phakomatosis: a report of four cases. Eur J Pediatr 144:290–294
Leonidas JC, Wolpert SM, Feingold M, et al (1979) Radiographic features of the linear nevus sebaceous syndrome. AJR 132:277–279
Fretzin DF, Lawrence MS (1968) The epidermal nevus syndrome. Cutis 4:1336–338
Sakuta R, Aikawa H, Takashima S, et al (1991) Epidermal nevus syndrome with hemimegalencephaly: neuropathological study. Brain Dev 13:260–265
Solomon LM, Fretzin DF, Dewald RL (1968) The epidermal nevus syndrome. Arch Dermatol 97:275–285
Happle R (1993) Mosaicism in human skin. Arch Dermatol 129:1460–1470
Chalhub EG, Volpe JJ, Gado MH (1975) Linear nevus sebaceous syndrome associated with porencephaly and nonfunctioning major cerebral sinuses. Neurology 25:857–860
Holden KR, Dekaban AS (1972) Neurological involvement in nevus unis lateralis and nevus linearis sebaceus. Neurology 22:879–887
Solomon LM, Esterley NB (1968) Epidermal and other congenital organoid nevi. Curr Probl Pediatr 6:3-56
Spranger J, Langer LO, Wiedemann HR (1974) Bone dysplasias. Fischer, Stuttgart, p 203
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Neumann, L.M., Scheer, I., Kunze, J. et al. Cerebral manifestations, hemihypertrophy and lymphoedema of one leg in a child with epidermal nevus syndrome (Schimmelpenning-Feuerstein-Mims). Pediatr Radiol 33, 637–640 (2003). https://doi.org/10.1007/s00247-003-0944-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-003-0944-3