• Vol. 28 No. 5, 744–748
  • 15 September 1999

Oral Manifestations of Schimmelpenning Syndrome: Case Report and Review of Literature

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ABSTRACT

Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities. Here, the SS is briefly reviewed, and the oral and dental manifestations are described in a patient whose medical findings were previously published and included severe hypophosphatemic rickets. Significant oral and dental features included papillomatous lesions of the gingiva, hemihyperplasia (hemihypertrophy) of the tongue, bone cysts, aplasia of teeth, enlarged pulp chambers, hypoplastic or absent enamel, and an odontodysplasia-like permanent tooth.


Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities, such as mental deficiency and seizures. Happle has shown that a clinical entity known as “epidermal nevus syndrome”, formerly used as an alternative term to SS, does not exist.

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