Labial salivary gland involvement in neonatal hemochromatosis: a report of 2 cases and review of literature

Neonatal hemochromatosis (NH) is a severe disease of fetal or perinatal onset, in which iron deposition occurs within hepatic and extrahepatic sites without involving the reticuloendothelial system. Labial minor salivary gland biopsy has been suggested as a diagnostic adjunct in patients suspected of having NH, as hemosiderin accumulates in acinar epithelial cells. Prior to this salivary gland pathology, a diagnosis of NH was often delayed, rendered only after the usual causes of neonatal liver failure had been excluded. Recent studies have shown that early diagnosis and treatment can improve survival. Few cases of salivary gland hemosiderosis in NH have been reported in the literature. A positive finding of salivary gland siderosis on biopsy will expedite care. We report 2 cases of NH, of which a labial salivary gland biopsy supported the diagnosis. The clinical and histological features are presented. The NH literature pertaining to labial salivary gland pathology is reviewed.