Facial granulomatous lymphoedema and syringomyelia

The term orofacial granulomatosis was introduced for orofacial lesions which resemble Crohn's disease clinically and histologically in patients without gastrointestinal disease. The Melkersson-Rosenthal syndrome and its oligosymptomatic form Meischers granulomatous cheilitis are usually felt to be part of the same spectrum of disease. Oedema usually involves tissues of the lower half of the face. We describe a patient with histological changes consistent with those found in orofacial granulomatosis, but with isolated localized oedema of the right periorbital tissues. The patient also had syringomyelia which has not previously been described in association with orofacial granulomatosis.