Is the distribution of dermal neurofibromas in neurofibromatosis type 1 (NF1) related to the pattern of the skin surface temperature?

The formation of dermal neurofibromas is a hallmark of the neurofibromatosis type 1 (NF1). A total loss of the NF1 gene product by stochastic events inactivating the wild type allele in Schwann cells should precede the development of neurofibromas. Dermal neurofibromas tend to be located mainly on t...

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Bibliographic Details
Published inEJD. European journal of dermatology Vol. 11; no. 6; p. 521
Main Authors Kaufmann, D, Tinschert, S, Algermissen, B
Format Journal Article
LanguageEnglish
Published France 01.11.2001
Subjects
Adolescent
Adult
Aged
Aged, 80 and over
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Male
Middle Aged
Neurofibroma - genetics
Neurofibroma - pathology
Neurofibromatosis 1 - genetics
Neurofibromatosis 1 - pathology
Skin Temperature
Thermography - methods
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Summary:The formation of dermal neurofibromas is a hallmark of the neurofibromatosis type 1 (NF1). A total loss of the NF1 gene product by stochastic events inactivating the wild type allele in Schwann cells should precede the development of neurofibromas. Dermal neurofibromas tend to be located mainly on the surface of the trunk and not in the body periphery. This distribution partly resembles the density of sensitive nerve endings in the epidermis. Our hypothesis is that a better correlation concerns the pattern of normal body surface temperature. According to our clinical observations we assume that in skin areas with higher temperatures the number of visible dermal neurofibromas is higher than in colder areas such as the arms/legs or nose. It is known that differences in temperature are able to determine differentiation. We suggest that the regulation of skin temperature is also involved in the formation of NF1 dermal neurofibromas and is related to the intrafamilial variability in NF1.
ISSN:1167-1122