Linear Segmental Neurofibromatosis in an L5 Distribution

2012 ◽  
Vol 16 (6) ◽  
pp. 436-437
Author(s):  
Nicole Umanoff ◽  
Kate V. Viola ◽  
Jena Auerbach ◽  
Steven R. Cohen

Background and Objective: Neurofibromatosis (NF) is a neurocutaneous disorder with seven subtypes;segmental NF-1 is characterized by neurofibromas and or café-au-lait macules in one or more dermatomes, primarily the thorax and abdomen. We present the rare case of a 36-year-old African-American female with isolated, multiple soft nodules in a linear configuration on the plantar and dorsal surfaces of the left foot. Conclusion: This is the first report of isolated segmental NF-1 in a unique linear pattern confined to the L5 dermatome. Contexte et objectif: La neurofibromatose (NF) est un trouble neurocutané, qui se divise en sept sous-types; celle de type 1 (NF-1) segmentaire se caractérise par des neurofibromes ou des macules de couleur « café au lait » dans l'un ou plusieurs dermatomes, surtout sur le thorax et l'abdomen. Voici le rare cas d'une femme afro-américaine, de 36 ans, qui présente de nombreux nodules mous, isolés, suivant un trajet linéaire sur les faces plantaire et dorsale du pied gauche. Conclusion: Il s'agit du premier cas signalé de NF-1 segmentaire, isolée, suivant un trajet linéaire particulier, limité au dermatome L5.

2000 ◽  
Vol 95 (9) ◽  
pp. 2580-2580 ◽  
Author(s):  
Hitender Jain ◽  
M.A. Albornoz ◽  
J. Collazo ◽  
J.M. Greenblatt ◽  
J.M. Draganescu ◽  
...  

2018 ◽  
Vol 29 (1) ◽  
pp. 41-47 ◽  
Author(s):  
Stella Ioannou ◽  
Maciej Henneberg

Congenital syphilis is a disease recognized for interfering with odontogenesis, producing specificdental characteristics including Hutchinson’s incisor, Moon’s molar, Fournier’s molar and mulberry molar,while its past treatments including mercury are known to affect amelogenesis. Supernumerary teeth, mainly associatedwith syndromes, are not commonly found in cases of congenital syphilis. A rare case of congenitalsyphilis in an individual (P000707) treated with mercury and a mandibular left fourth molar with normal morphologyis presented.Materials and Methods: During a systematic examination of 28 skeletons with treponemal disease at the Smithsonianmuseum in Washington, DC, a supernumerary mandibular distomolar in one individual (P000707) wasrevealed.Results: P000707 was an African American female, 26 years of age. Dentition showed severe enamel hypoplasiaof the maxillary and mandibular incisors, left canine, and upper first molars, consistent with the effects of treatmentof congenital syphilis by mercurial compounds. Crown of the left mandibular distomolar has typical molarmorphology but is smaller in size than other permanent molars. Arrangement of grooves resembles the +4 pattern,but is complex due to crenulation. Oblique x-ray revealed that the fourth molar had one root with a pulpchamber extending towards the apex, suggesting taurodontism. No other distomolar teeth were present.Conclusions: Congenital syphilis and treatment containing mercury may not influence the development of supernumeraryteeth due to: (1) the age at which the development of the fourth molar takes place, (2) the stage ofthe infection at the time of development and (3) the age at which treatments containing mercury are administeredto patients with congenital syphilis.


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