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Histopathology and electron and immunofluorescence microscopy of gingivitis granulomatosa associated with glossitis and cheilitis in a case of Anderson-Fabry disease.

A 17-year-old white boy with signs, symptoms, and family history of angiokeratoma corporis diffusum universale, Anderson-Fabry disease (AFD), developed recurrent and then persistent swelling of both lips, erythematous hyperplastic gingivae, and a pebbled tongue. Positive blood findings were raised serum IgE, decreased T-cell level, and increased B-cell level. Histopathology of the gingiva showed noncaseating granulomas with multinucleate giant cells containing Schaumann bodies and large plasma-cell infiltrates in which immunofluorescence demonstrated immune globulins of several classes. Electron microscopy and histochemistry demonstrated ceramide in the vasculature. No glycolipid was found in the macrophages or giant cells of the granulomas which, in contrast, resembled sarcoid reactions. Plasma cells with Russell bodies and immune reaction-induced degranulation of mast cells were also identified. The pathogenesis of the oral findings possibly relates to altered immune reactivity associated with damage to the microvasculature analogous to that in Melkersson-Rosenthal syndrome.

Adolescent↗

Glossitis and other tongue disorders.

Patients frequently present complaining of tongue abnormalities. Knowledge of normal tongue anaomy an d architecture enable the clinician t odifferentiate variations of normal from abnormal conditions. Many tongue conditions are benign and. require reassurance and explanation, with little to no treatment. Others can signify systemic disorders. Examination of the tongue is an integral part of a complete physical examination. Recognizing the disorders of the tongue that are benign and do not require treatment or further evaluation prevents unnecessary testing for the patient. Careful evaluation of the tongue may provide valuable clues to a systemic disorder.

Glossitis↗