Case report: Infantile systemic hyalinosis: A dental perspective

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 (hyperpig- mentation, subcutaneous nodules), malnutrition resulting from diarrhoea, gingival, labial and buccal hypertrophy. Case Report: The phenotype characteristics of infan- tile 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 hyper- trophy completely covering maxilary and mandibular teeth treatment. The gingival hypertrophy was surgically treated by gingivectomy under general anaesthesia. Follow- Up: The patient showed a full constellation of clinical manifestations of the disease. Despite the surgical inter- vention no improvement in oral hygiene was observed. Conclusions: Surgical treatment of the gingival hyper- trophy was the treatment of choice.