Cheilitis Granomatosa

Abstract

To increase awareness of the unusual manifestations of extraintestinal Crohn's disease by reporting a solitary granulomatous lesion of the lower lip in a patient with Crohn's colitis. A biopsy of the lower lip was taken from a patient with active Crohn's disease who complained of swelling of the lips. A 53 year old female with long standing Crohn's disease (CD) described several months of discomfort and swelling of the lower lip. Physical examination revealed a fissure of the median line of the lower lip with swelling, that, on palpation, had a superficial granular texture and rubbery non pitting edema. Her serum chemistries, liver enzymes, CBC and CRP, were normal. Biopsy of the lip revealed small noncaseating granulomas, subepidermal lymphaedema, and inflammatory infiltrates. Cheilitis granulomatosa (CG) or Miescher's cheilitis, swelling of the lips, was first described in 1945, but its causes remain unknown. Allergic reactions to cobalt or food additives have been implicated. CG may be considered a subtype of oroafacial granulomatosis (OFG), which is the chronic swelling of the lips and the lower half of the face with oral ulcerations and hyperplastic gingivitis. Alone it can be viewed as a monosymptomatic variant of the Melkersson- Rosenthal syndrome (facial paralysis, swelling of the face and lips and furrows in the tongue). It has also been noted in systemic diseases sarcoidosis and CD. Only.5% of patients with CD ever develop CG. Pathological specimens may reveal non-necrotizing granulomas, edema, lymphangiectasia, and/or perivascular lymphocytic infiltration. Nevertheless, the diagnosis is made clinically based on a patient's history, symptoms and physical examination. Some authors believe a topical anesthetic with systemic corticosteroids is the most effective, while others recommend intralesional steroids. Jenss recommended local steroid therapy in patients on low dose systemic steroids if OFG occurs without gastrointestinal signs. Surgical intervention may only be considered if the disease in the quiescent stage and there is pronounced disfigurement. Although pathologically, the two entities appear grossly similar, it is unclear what the relationship is between cheilitis granulomatosa and Crohn's disease. Further immunological, microbiological and histological testing must be performed to delineate the similarities and differences between these two entities and further define their relationship.