Case report: Infantile systemic hyalinosis: a dental perspective
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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 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 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 intervention no improvement in oral hygiene was observed. CONCLUSIONS: Surgical treatment of the gingival hypertrophy was the treatment of choice.
KeywordsInfantile systemic hyalinosis non-steroidal anti-inflammatory drugs medium chain triglyceridies
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