title: [Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases].
abstract: BACKGROUND
  Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome.
  
  
  METHODS
  The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage.
  
  
  CONCLUSIONS
  Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas.
authors.affiliation: Service de dermatologie, hôpital Charles-Nicolle, Tunis, Tunisie. fekih.nadia@planet.tn
authors.forename: Nadia
authors.initials: N
authors.lastname: El Fekih
authors.forename: Anis
authors.initials: A
authors.lastname: Mahfoudh
authors.forename: Samy
authors.initials: S
authors.lastname: Zekri
authors.forename: Monia
authors.initials: M
authors.lastname: Kharfi
authors.forename: Bécima
authors.initials: B
authors.lastname: Fazaa
authors.forename: Mohamed Habib
authors.initials: MH
authors.lastname: Jaafoura
authors.forename: Mohamed Ridha
authors.initials: MR
authors.lastname: Kamoun
mesh_terms: D000293:Adolescent; D001768:Blister / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology*; D002648:Child; D003241:Consanguinity; D020405:Dermis / Q000648:ultrastructure; D003937:Diagnosis, Differential; D004817:Epidermis / Q000648:ultrastructure; D004820:Epidermolysis Bullosa / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology*; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D008854:Microscopy, Electron; D010510:Periodontal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology*; D010787:Photosensitivity Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology*; D014416:Tunisia / Q000453:epidemiology; D055815:Young Adult