Skip to main content

Cerebral manifestations, hemihypertrophy and lymphoedema of one leg in a child with epidermal nevus syndrome (Schimmelpenning-Feuerstein-Mims)

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, access via your institution.

Fig. 1.
Fig. 2a, b.
Fig. 3a–d.

References

  1. 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

  2. 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

    CAS  PubMed  Google Scholar 

  3. Happle R (1995) Epidermal nevus syndromes. Semin Dermatol 14:111–121

    CAS  PubMed  Google Scholar 

  4. Schimmelpenning GW (1983) Langjährige verlaufskontrolle einer organoiden nävusphakomatose (Schimmelpenning-Feuerstein-Mims). Fortschr Röntgenstr 139:63–67

    Google Scholar 

  5. 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

    CAS  PubMed  Google Scholar 

  6. 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

    CAS  PubMed  Google Scholar 

  7. 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

    CAS  PubMed  Google Scholar 

  8. Leonidas JC, Wolpert SM, Feingold M, et al (1979) Radiographic features of the linear nevus sebaceous syndrome. AJR 132:277–279

    CAS  Google Scholar 

  9. Fretzin DF, Lawrence MS (1968) The epidermal nevus syndrome. Cutis 4:1336–338

    Google Scholar 

  10. Sakuta R, Aikawa H, Takashima S, et al (1991) Epidermal nevus syndrome with hemimegalencephaly: neuropathological study. Brain Dev 13:260–265

    CAS  PubMed  Google Scholar 

  11. Solomon LM, Fretzin DF, Dewald RL (1968) The epidermal nevus syndrome. Arch Dermatol 97:275–285

    Google Scholar 

  12. Happle R (1993) Mosaicism in human skin. Arch Dermatol 129:1460–1470

    Article  CAS  PubMed  Google Scholar 

  13. Chalhub EG, Volpe JJ, Gado MH (1975) Linear nevus sebaceous syndrome associated with porencephaly and nonfunctioning major cerebral sinuses. Neurology 25:857–860

    CAS  PubMed  Google Scholar 

  14. Holden KR, Dekaban AS (1972) Neurological involvement in nevus unis lateralis and nevus linearis sebaceus. Neurology 22:879–887

    CAS  PubMed  Google Scholar 

  15. Solomon LM, Esterley NB (1968) Epidermal and other congenital organoid nevi. Curr Probl Pediatr 6:3-56

    Google Scholar 

  16. Spranger J, Langer LO, Wiedemann HR (1974) Bone dysplasias. Fischer, Stuttgart, p 203

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Luitgard M. Neumann.

Rights and permissions

Reprints 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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-003-0944-3

Keywords

  • Epidermal nevus syndrome (Schimmelpenning-Feuerstein-Mims)
  • Fibrous dysplasia
  • Hemihypertrophy
  • Lymphoedema
  • MRI