Gustav Schimmelpenning and the Syndrome Bearing His Name

Abstract

Gustav Schimmelpenning was born in 1928 in Oldenburg (Germany). From 1971 until 1994 he was head of the Department of Psychiatry at the University of Kiel. In 1957, while training in neurology and psychiatry, he comprehensively described a case of sebaceous nevus involving the head, with ipsilateral ocular lesions including coloboma of the upper lid, increased density of cranial bones, epileptic seizures and mental retardation. He concluded that this combination of anomalies represented a new ‘phacomatosis’. Subsequently this phenotype was reported by other authors under many different names, such as ‘Schimmelpenning syndrome’, ‘Feuerstein-Mims syndrome’, ‘Schimmelpenning-Feuerstein-Mims syndrome’, ‘epidermal nevus syndrome’, ‘Solomon syndrome’, ‘linear sebaceous nevus syndrome’, ‘organoid nevus phacomatosis’, or ‘Jadassohn nevus phacomatosis’. As a consequence of this confusing terminology, Schimmelpenning syndrome even has two different OMIM entries (no. 163200 and no. 165630). The term ‘Schimmelpenning syndrome’ is both historically justified and practically sufficient to distinguish this phenotype from other epidermal nevus syndromes.