title: Case report: Infantile systemic hyalinosis: a dental perspective.
abstract: BACKGROUND
  Infantile systemic hyalinosis is a rare genetic disorder which involves accumulation of hyaline in the skin, bones, mucous membranes, and occasionally, also in internal organs. The major manifestations include painful articular contractures, cutaneous lesions (hyperpigmentation, subcutaneous nodules), malnutrition resulting from diarrhoea, gingival, labial and buccal hypertrophy.
  
  
  METHODS
  The phenotype characteristics of infantile systemic hyalinosis (ISH) in a two year old boy were present. The characteristics of flattered occiput, limited limb movements and articular abnormalities of elbows and knees. Dental findings showed excessive gingival hypertrophy completely covering maxillary and mandibular teeth treatment. The gingival hypertrophy was surgically treated by gingivectomy under general anaesthesia. FOLLOWUP: The patient showed a full constellation of clinical manifestations of the disease. Despite the surgical intervention no improvement in oral hygiene was observed.
  
  
  CONCLUSIONS
  Surgical treatment of the gingival hypertrophy was the treatment of choice.
authors.affiliation: Department of Pediatric Dentistry, Medical University of Warsaw, ul. Miodowa 18 Warsaw 00-246, Warsaw, Poland. do-k@o2.pl
authors.forename: D
authors.initials: D
authors.lastname: Olczak-Kowalczyk
authors.forename: E
authors.initials: E
authors.lastname: Krasuska-Slawinska
authors.forename: D
authors.initials: D
authors.lastname: Rokicki
authors.forename: M
authors.initials: M
authors.lastname: Pronicki
mesh_terms: D002675:Child, Preschool; D005886:Gingival Hypertrophy / Q000209:etiology* / Q000601:surgery*; D005890:Gingivectomy; D006801:Humans; D057770:Hyaline Fibromatosis Syndrome / Q000150:complications*; D008297:Male; D009910:Oral Hygiene / Q000193:education