• Users Online: 353
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
CASE REPORT
Year : 2020  |  Volume : 4  |  Issue : 1  |  Page : 46-49

Co-Occurrence of neurofibromatosis-1 with urticaria pigmentosa: A Coincidental association?


Department of Dermatology, Shri B. M. Patil Medical College, Hospital and Research Centre, BLDE University, Bijapur, Karnataka, India

Correspondence Address:
Arun C Inamadar
Department of Dermatology, Shri B. M. Patil Medical College, Hospital and Research Centre, BLDE University, Bijapur - 586 103, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_37_18

Rights and Permissions

Neurofibromatosis-1 (NF-1) is a neuroectodermal abnormality characterized by multiple neurofibromas, café-au-lait macules, axillary freckling, and Lisch nodules. Urticaria pigmentosa (UP) is a maculopapular cutaneous mastocytosis due to mast cell hyperplasia. We report a case of NF-1 associated with UP. A 19-year-old male presented with multiple neurofibromas, café-au-lait macules, axillary freckling, and Lisch nodules. He also had UP lesions with positive Darier's sign. A clinical diagnosis of NF-1 with UP was made and confirmed histopathologically. Mastocytosis exhibits mast cell hyperplasia due to C-kit gene mutation. Mast cell mediatiors directly contribute to neurofibroma growth. C-kit receptor abnormality is implicated in the formation of neurofibromas as well as in UP. With possible interconnected underlying pathology of mast cell hyperplasia and increased mast cell mediators in both UP and NF-1, a possible association of NF and UP beyond simple coincidence should be considered.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed566    
    Printed45    
    Emailed0    
    PDF Downloaded56    
    Comments [Add]    

Recommend this journal