title: [Congenital insensitivity to pain: difficulty of management].
abstract: BACKGROUND
  Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare disorder, most often of genetic origin.
  
  
  METHODS
  The authors present the case of two siblings, 10 and 13 years old, both followed-up since the age of 2 for CIPA diagnosed after discovering insensitivity to pain during iterative falls, burns, and of severe oro-digital self-mutilating behavior. Sural nerve biopsy and an electromyogram confirmed the diagnosis.
  
  
  CONCLUSIONS
  CIPA with anhidrosis is a very rare disease. It is characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and sometimes mental retardation. Complications of this insensitivity (non-treated fractures, burns, and oro-digital mutilation) may be lethal. Treatment remains preventive. The patient must observe a very strict hygiene. Prevention for maxillofacial involvement consists in breaking the cycle of oral self-mutilation.
authors.affiliation: Service d'Otorhinolaryngologie et de Chirurgie Maxillo-Faciale, Hôpital de Spécialités, Rabat, Maroc.
authors.forename: H
authors.initials: H
authors.lastname: Benhalima
authors.forename: S
authors.initials: S
authors.lastname: Kerrary
authors.forename: D
authors.initials: D
authors.lastname: Kamal
authors.forename: M
authors.initials: M
authors.lastname: Boulaich
authors.forename: M
authors.initials: M
authors.lastname: Kzadri
mesh_terms: D000293:Adolescent; D002648:Child; D037001:Directive Counseling; D005260:Female; D006801:Humans; D007007:Hypohidrosis / Q000150:complications / Q000175:diagnosis; D008607:Intellectual Disability / Q000150:complications / Q000175:diagnosis; D008297:Male; D000699:Pain Insensitivity, Congenital / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D012652:Self Mutilation / Q000150:complications / Q000517:prevention & control; D035781:Siblings; D009057:Stomatognathic Diseases / Q000150:complications / Q000175:diagnosis