[Oral manifestations of Letterer-Siwe disease].

In Letterer-Siwe disease, oral and dental pathology is an unusual occurrence. Failure to consider this condition in patients with gingival inflammation, necrosis, or premature eruption of teeth may lead to delay in diagnosis. We present two cases with these features. Case Reports Case 1. This boy was aged 4 months when he developed sore, swollen gums with white necrotic areas. He was treated for 5 months for stomatitis. At age 6 months he had bilateral discharging otitis and a rash over his back; these both cleared over the next 2 months. He was seen at this hospital aged 10 months, thin, miserable, and underweight. The gingivae over upper and lower jaws were swollen and necrotic with premature eruption of teeth floating in a mass of granulomatous tissue (Fig.).. _. S r FIG.-Oral cavity showed hypertrophied and necrotic gingivae with eruption of teeth. He had crusted erythematous scalp lesions. X-ray showed an osteolytic lesion involving the alveolar margin and the body of the right mandible, extending back to the ascending ramus. Chest x-ray showed fine diffuse opacities in both lung fields. Biopsy showed gingival, peridontal, and bony infiltration with histiocytes and eosinophils compatible with the diagnosis of Letterer-Siwe disease. Treatment was started with vinblastine 0 1 mg/kg per week and prednisone 2 mg/kg per day. At follow-up 3 months later the gingivae appeared normal with no hypertrophy or ulceration, and his chest x-ray showed only very minimal scattered opacification. At the age of 2 months he was referred to hospital with the combination of difficulty in feeding related to oral ulceration and the persistence of the rash present at birth. He had swollen, necrotic, ulcerated gums, and over the next few weeks developed premature eruption of teeth floating in a mass of soft granulomatous tissue. There were also widespread pityriasiform-like skin lesions which left dermal scarring on healing. He later developed a discharging right ear. Skull x-ray showed translucent areas in the parietal bones. X-ray of the mandible showed alveolar absorption around 1/1 and periosteal reaction around the horizontal rami. Gingival biopsy performed when aged 3 months showed grey-white necrotic tissue and histology confirmed the diagnosis of Letterer-Siwe disease: this was also confirmed on skin biopsy. Treatment was started with vinblastine 0 1 mg/kg per week and prednisone 2 mg/kg per day. He rapidly deteriorated despite treatment, with the development of hepatosplenomegaly and obstructive jaundice, and died aged 5 months. Discussion …