| Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. | |
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MedLine Citation:
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PMID: 11872407 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Speckled lentiginous nevus syndrome, a so far unrecognized cutaneous phenotype associated with neurological anomalies, is postulated on the basis of the following arguments. Phacomatosis pigmentokeratotica represents a twin spot phenomenon. One isolated half of this complex phenotype is observed rather often in the form of Schimmelpenning syndrome, whereas the other half of this twin nevus syndrome consists of a speckled lentiginous nevus associated with various neurological abnormalities incompatible with Schimmelpenning syndrome, such as hyperhidrosis, muscular weakness and dysesthesia. This second component of phacomatosis pigmentokeratotica may likewise occur separately. For the association of speckled lentiginous nevus with hyperhidrosis, muscular weakness, dysesthesia or other neurological abnormalities, the term "speckled lentiginous nevus syndrome" is proposed. Some case reports that may be categorized as examples of this new syndrome are reviewed. The postulated new phenotype is tentatively categorized as a paradominant trait. Future clinical studies will probably confirm the existence of speckled lentiginous nevus syndrome as a distinct neurocutaneous phenotype. |
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Authors:
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Rudolf Happle |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: European journal of dermatology : EJD Volume: 12 ISSN: 1167-1122 ISO Abbreviation: Eur J Dermatol Publication Date: 2002 Mar-Apr |
Date Detail:
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Created Date: 2002-03-01 Completed Date: 2002-04-25 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9206420 Medline TA: Eur J Dermatol Country: France |
Other Details:
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Languages: eng Pagination: 133-5 Citation Subset: IM |
Affiliation:
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Department of Dermatology, Philipp University of Marburg, Deutschhausstrasse 9, D-35033 Marburg, Germany. happle@mailer.uni-marburg.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cafe-au-Lait Spots
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diagnosis*,
genetics Diagnosis, Differential Humans Neurocutaneous Syndromes / diagnosis*, genetics Nevus, Pigmented / diagnosis*, genetics Phenotype Skin Neoplasms / diagnosis*, genetics Syndrome Terminology as Topic |
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