Psychoneurocutaneous medicine: past, present and future.

Psychoneurocutaneous medicine is an important and rapidly growing field. It is an integrative science created to address the interaction between the mind, nervous system and skin. Four major fields are involved: psychiatry, psychology, neurology and dermatology. The history of psychoneurocutaneous medicine is old and fascinating. Even though it is a young science only established in the last century, allusions to the nervous system and diseases of the skin can be traced back thousands of years in the literature. Characteristically, the last two centuries were of special importance for the development of this field in Europe, lately in America, with great effort placed on research and publications. More recently, the creation of associations, work groups and the merging of subspecialties dedicated exclusively to study the psychological impact of skin disorders in subpopulations of patients have helped to give momentum to psychoneurocutaneous medicine. Further development of this field will shed light on the appropriate diagnosis and treatment of psychodermatologic disorders, enhancing the functionality and quality of life of patients. This paper presents a summary of the most influential facts in the history of psychoneurocutaneous medicine, its present and future perspectives.

Neurocutaneous melanocytosis.

Neurocutaneous melanosis or neurocutaneous melanocytosis is a rare sporadic congenital disorder characterized by the presence of giant and/or multiple satellite congenital melanocytic nevi in the skin and benign melanocytic pigmentation of the leptomeninges. These two defining features were recognized more than a century ago. A third characteristic feature is proliferative nodules arising from giant nevi. The etiology is unknown, but neurocutaneous melanosis is considered a developmental disorder of melanocyte precursors from neural crest. The distinctive unique distribution of the congenital giant nevi that gives a "garment" appearance is also an expression of the neural crest. The neurological manifestations often appear in infancy.The special association of neurocutaneous melanosis with Dandy-Walker malformation complex may be explained by a common pathogenesis. Mortality in infancy and childhood is high.

Gustav Schimmelpenning and the syndrome bearing his name.

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.

The sebaceous nevus as part of the Schimmelpenning-Feuerstein-Mims Syndrome--an obvious phacomatosis first documented in 1927.

The sebaceous nevus is a common nevus and can be easily diagnosed because of its typical rough fatty surface due to its amount of sebaceous glands. In some rare cases, the sebaceous nevus is part of a genetic disorder, the Schimmelpenning-Feuerstein-Mims (SFM) syndrome. If the SFM syndrome is suspected, further investigation is necessary, because multiple organ involvement is highly likely. We suggest that diagnosis of the SFM syndrome is simple, considering the special linear arrangement of sebaceous nevi in cases of SFM syndrome.