Can obstructive intralymphatic granulomas be the cause of cheilitis granulomatosa?

Cheilitis granulomatosa (ChG), also known as Miescher's cheilitis, is an uncommon, immunologically mediated nonnecrotizing granulomatous inflammatory disease characterized by recurrent, painless swelling of the lips. The aim of the study was a pathomorphological and immunohistochemical assessment of cases clinically classified as ChG to investigate potential pathological mechanisms of ChG symptoms and to verify the hypothesis of intravascular granulomas as a cause of lymphatic vessel obstruction and localized edema in ChG. We report 6 patients with ChG who clinically presented localized edema of the lips. Lip biopsy with pathomorphological and immunohistochemical examination was performed in all cases. We found discrete, non-necrotizing granulomas which were adjacent to numerous blood and lymphatic vessels. The lumen of lymphatic channels was dilated and was either empty or contained lymph and few macrophages or was completely occluded by nearby granulomas. All patients demonstrated a characteristic pattern of lymphangiectasia and perivascular lymphatic aggregates with evidence of non-necrotizing granulomas. None manifested intralymphatic granulomas. These results do not support the view that lymphatic vessel obstruction is caused by intravascular histiocytic granulomas described as the main part in the etiology of lymphatic edema in ChG. However, perivascular granulomas and dilation of lymphatic vessels confirm presence of inflammatory lymphostasis in all studied cases of ChG.