ABSTRACT
Cheilitis granulomatosa (CG) is a subset of orofacial granulomatosis (OFG) and considered to be a monosymptomatic form of Melkersson-Rosenthal syndrome (MRS), which is characterized by the triad of chronic lip swelling, facial paralysis, and fissured tongue. The labial swelling is thought to be associated with an orofacial swelling, which affects the chin, cheeks, and oral mucosa. Histologically, it is distinguished by noncaseating granulomas consisting of lymphohistiocytes and giant cells. Although systemic steroids with or without intralesional triamcinolone injections are the mainstay of treatment, and various agents have been proposed for this rare disease, no successful treatment modality has been reported in the literature yet. Herein, we present our experience with three different CG cases, which showed a varied level of positive response to the combination therapy.
Subject(s)
Cheek , Cheilitis , Chin , Facial Paralysis , Giant Cells , Granuloma , Granulomatosis, Orofacial , Lip , Melkersson-Rosenthal Syndrome , Mouth Mucosa , Rare Diseases , Steroids , Tongue, Fissured , TriamcinoloneABSTRACT
Cheilitis granulomatosa, a rare disease of unknown cause, is characterized clinically by recurrent or persistent swelling of the lip. Histopathologically, the dermis contains non-necrotizing granuloma, lymphangiectasia, and perivascular lymphocytic infiltration. Treatment of cheilitis granulomatosa is difficult, due to its unknown etiology. Several therapeutic methods have been attempted, including systemic and intralesional corticosteroids, oral metronidazole, non-steroidal anti-inflammatory drugs (NSAIDs), hydroxychloroquine, and surgical reduction, but unfortunately no treatment method has yet proven reliable.
Subject(s)
Adrenal Cortex Hormones , Cheilitis , Dermis , Granuloma , Hydroxychloroquine , Lip , Melkersson-Rosenthal Syndrome , Methods , Metronidazole , Minocycline , Rare DiseasesABSTRACT
Cheilitis granulomatosa, a rare disorder of unknown origin, is characterized by recurrent and persistent labial swelling. It is a monosymptomatic form of Melkersson-Rosenthal syndrome. Non-necrotizing granulomatous inflammation is seen at histopathologic examination. We report a 44-year-old woman with a month history of gradual swelling on her upper lip. We treated successfully with oral metronidazole medication for 6 months, although she had showed a poor response in oral prednisolone, antibiotics, several intralesional triamcinolone injection therapy.
Subject(s)
Female , HumansABSTRACT
Melkersson-Rosenthal syndrome is a rare condition and clinical diagnosis was established on the basis of the triad: facial or lip edema, peripheral facial palsy and scrotal or plicated tongue. Labial swelling(Cheilitis granulomatosa) is the most common feature of this syndrome complex. The lips may enlarge up to three times their normal size, resulting in aesthetic deformity and functional disability. The condition appears to be a granulomatous disorder causing edema and inflammation of the soft tissues of the face, lips, oral cavity and particularly, the facial nerve. Traditional medical interventions are only marginally successful in treating this syndrome. We report the case of a young man with cheilitis granulomatosa as a manifestation of Melkersson-Rosenthal syndrome successfully treated by transverse and vertical star-shaped resection with a transmodiolar labial suspension suture at mouth angle. Our result shows symmetry of the lip, a normalized anterior projection, dimensional harmony between upper and lower lip and no disfiguring cicatrices.
Subject(s)
Congenital Abnormalities , Diagnosis , Edema , Facial Nerve , Facial Paralysis , Inflammation , Lip , Melkersson-Rosenthal Syndrome , Mouth , Sutures , TongueABSTRACT
Cheilitis granulomatosa is a rare disorder of uncertain etiology and characterized by a recurrent orofacial swelling, which was described by Miescher in 1945 for the first time. Symptoms usually manifest during adolescence and initially transient, but subsequently a chronic progressive disability develops. No efficient mode of therapy is yet available. A 71-year-old woman presented with a prominent upper lip swelling which had persisted for more than 6 years. There were no fissuring of the tongue and facial palsy. Oral prednisone was instituted for 6 months with moderate clinical improvement. 2 years later, multiple biopsies were performed at the hard palate, gingiva, and inferior turbinate. And the specimens from the gingiva and inferior turbinate showed scattered non-caseating granulomas, consistent with 'Cheilitis Granulomatosa'. We present here our experience with long-term trial of oral corticosteroid in a 71-year-old woman with cheilitis granulomatosa who had suffered recurrent labial swelling.
Subject(s)
Adolescent , Aged , Female , Humans , Biopsy , Cheilitis , Facial Paralysis , Gingiva , Granuloma , Lip , Melkersson-Rosenthal Syndrome , Palate, Hard , Prednisone , Tongue , TurbinatesABSTRACT
Cheilitis granulomatosa, which is a rare inflammatory disorder of unknown origin, was described by Miescher in 1945. A 31-year-old man presented with swelling of both lips without any subjective symptom that had been present for 1 year. The patient had the repeated episodes of upper lip which would resolve in days to weeks that had ended up involving lower lip and eventually persistent. Histopathological study revealed a noncaseating granulomatous inflammation with perivascular lymphocytic infiltration. The patient was treated with systemic steroids and received cheiloplasty. We describe a case of cheilitis granulomatosa improved with systemic steroids and the cheiloplasty.
Subject(s)
Adult , Humans , Cheilitis , Inflammation , Lip , Melkersson-Rosenthal Syndrome , SteroidsABSTRACT
Cheilitis granulomatosa as a monosymtomatic form of the Melkersson-Rosenthal sydrome, was charaterized by reccurrent or persistent edema in the orofacial lesion that could not be explained on the basis of infections, systemic, malignant, or connective tissue disorders. Herein we report two cases of cheilitis granulomatosa which have been treated with triamcinolone acetonide intralesional injection, hydroxychloroquine 400mg per day. After a short course of systemic steroids the swelling of the lip improved.
Subject(s)
Cheilitis , Connective Tissue , Edema , Hydroxychloroquine , Injections, Intralesional , Lip , Melkersson-Rosenthal Syndrome , Steroids , Triamcinolone AcetonideABSTRACT
A 43-year-old female had diffuse swelling with erythematous papules on the lower lip and an erythematous patch on the chin for 1 year. The lesions were nonpururitic, non-tender and odontogenic infection was combined. A Histopathological study of the biopsy specimen of the chin showed noncaseating epithelioid granuloma through the entire dermis. Special stains showed no evidence of fungal organism or acid fast bacilli. Theses clinical and histopathologic findings indicated the diagnosis of cheilitis granulomatosa. Odontogenic infectious sources were removed and antibiotics and dapsone oral medication have been used and the lesions showed improvement.
Subject(s)
Adult , Female , Humans , Anti-Bacterial Agents , Biopsy , Chin , Coloring Agents , Dapsone , Dermis , Diagnosis , Granuloma , Lip , Melkersson-Rosenthal SyndromeABSTRACT
We report herein two cases of cheilitis granulcmatosa which were beneficial effects with intralesional triamcinolone acetonide. Casc 1, 27-year-old, showed both lip swelling with moderate scrotal tongue for 7 years and Case 2, 50-year-old, -had lower lip swelling for 1. 4 years. Histopathologic findings show lymphohistiocytic infiltration in the dermis with focal tuberculoid granuloma in case 1 and ill-defined granuloma in case 2.