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.
Cheek ; Cheilitis ; Chin ; Facial Paralysis ; Giant Cells ; Granuloma ; Granulomatosis, Orofacial ; Lip ; Melkersson-Rosenthal Syndrome ; Mouth Mucosa ; Rare Diseases ; Steroids ; Tongue, Fissured ; Triamcinolone

Melkersson-Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child's lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn's disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn's disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS.
Biopsy ; Child* ; Crohn Disease ; Diagnosis ; Edema ; Facial Paralysis ; Follow-Up Studies ; Genetic Testing ; Genitalia* ; Granuloma ; Humans ; Lip ; Lymphatic Vessels ; Male* ; Melkersson-Rosenthal Syndrome* ; Penis ; Tongue, Fissured

Melkersson-Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child's lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn's disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn's disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS.
Biopsy ; Child* ; Crohn Disease ; Diagnosis ; Edema ; Facial Paralysis ; Follow-Up Studies ; Genetic Testing ; Genitalia* ; Granuloma ; Humans ; Lip ; Lymphatic Vessels ; Male* ; Melkersson-Rosenthal Syndrome* ; Penis ; Tongue, Fissured

Geographic tongue, also called benign migratory glossitis, is a common and superficial benign inflammatory disorder that affects the tongue epithelium. The majority of geographic tongue lesions typically manifest as irregular central erythematous patches. These lesions, which are caused by the loss of filiform papillae, are defined by an elevated whitish band-like border that can change location, size, and pattern over a period of time. Histological observations of the oral mucosa affected by geographic tongue revealed nonspecific inflammation. Some reports described cases of migratory stomatitis, wherein lesions simultaneously manifested on the extra lingual oral mucosa. This condition is also called ectopic geographic tongue, which is clinically and histologically similar to the type normally confined to the tongue. In most cases, patients are asymptomatic and do not require treatment. The condition may spontaneously exhibit periods of remission and exacerbation with good prognosis. The specific etiology of geographic tongue remains unknown. Geographic tongue is age-related and is prevalent among young individuals. Various etiological factors that have been suggested in literature include immunological factors, genetic factors, atopic or allergic tendency, emotional stress, tobacco consumption, hormonal disturbances, and zinc deficiency. Geographic tongue may coexist with other disorders, such as fissured tongue, psoriasis, diabetes mellitus, gastroin- testinal diseases, burning mouth syndrome, and Down syndrome. Experts currently disagree on whether geographic tongue is an oral manifestation of psoriasis. Moreover, some scholars suggest that geographic tongue is a prestage of fissured tongue. The objective of this review is to summarize current research on risk factors of geographic tongue.
Epithelium ; Female ; Glossitis, Benign Migratory ; Humans ; Mouth Mucosa ; Risk Factors ; Tongue ; Tongue, Fissured

Acromegaloid Facial Appearance syndrome is a very rare syndrome combining acromegaloid-like facial appearance, thickened lips and oral mucosa and acral enlargement. Progressive facial dysmorphism is characterized by a coarse facies, a long bulbous nose, high-arched eyebrows, and thickening of the lips, oral mucosa leading to exaggerated rugae and frenula, furrowed tongue and narrow palpebral fissures. We report a case of acromegaloid facial appearance syndrome in a 19-year-old male patient who presented with all the characteristic features of the syndrome along with previously unreported anomalies like dystrophic nails, postaxial polydactyly and incisal notching of teeth.
Acromegaly ; Eyebrows ; Facies ; Humans ; Hypertrichosis ; India ; Limb Deformities, Congenital ; Lip ; Male ; Mouth Mucosa ; Nails ; Nose ; Polydactyly ; Tongue, Fissured ; Tooth

BACKGROUND: Geographic tongue (GT) is an inflammatory disorder of the oral mucosa. The clinical characteristics of GT have not been studied in Korea. OBJECTIVE: The purpose of this study was to investigate the clinical characteristics, associated factors, and treatment outcomes of GT. METHODS: We reviewed the medical records and clinical photographs of 55 cases who had been diagnosed with GT during the last 5 years. RESULTS: Of the 55 cases, 15 were male and 40 were female. The average age of onset was 29.3 years, and the highest incidence occurred in the 10~19 age group. The clinical type without circinate border was found in 67.3% of the cases and the clinical type with circinate border in 32.7% of the cases. Fissured tongue was found in 40% of the cases. Except for the 11 asymptomatic cases, the rest of the cases complained of various symptoms related to pain, irritation, and sensory changes. Hot, spicy or salty food acted as an aggravating factor in 74.5% of the cases and fatigue or stress in 61.8% of the cases. The majority of our cases (80.0%) were treated with topical steroid or steroid gargle or with a combination of both, and improvement was observed in 75.0% of this group. CONCLUSION: This is the first clinical study of GT in Korean literature. This study showed differences with previous studies in the proportion of cases with symptoms and aggravating factors. This study also suggested that topical steroid or steroid gargle could be the treatment of choice for GT.
Age of Onset ; Fatigue ; Female ; Glossitis, Benign Migratory ; Humans ; Incidence ; Male ; Medical Records ; Mouth Mucosa ; Tongue, Fissured

Pyogenic granuloma is a benign vascular tumor related to trauma, infection, or hormonal changes. When it affects the oral cavity, the most frequent site is the gingiva and involvement of the tongue is very rare. Occurrence of pyogenic granuloma on the fissured tongue has not been reported yet. We present a rare case of pyogenic granuloma on the tongue in a 64 year old female patient in which the fissured tongue seemed to play important roles in the pathogenesis of occurrence of the pyogenic granuloma.
Female ; Gingiva ; Granuloma, Pyogenic* ; Humans ; Middle Aged ; Mouth ; Tongue ; Tongue, Fissured*

OBJECT AND METHOD: Minor physical anomalies(MPAs) are frequently seen in patients with schizophrenia. MPAs are considered to arise from the anomalous development of ectoderm-originated tissues in the developing fetus. Since the central nervous system originates from ectoderm, MPAs can be regarded as externally observable and objective indicators of the aberrant development which might have taken place in the central nervous system. To investigate whether MPAs are more frequent in schizophrenic patients, the frequencies of MPAs were compared between schizophrenic patients and normal controls. Total 245 schizophrenic patients diagnosed with DSM-IV(male : 158, female : 87), and 418 normal control subjects(male : 216, female : 202) were included in this study. The MPAs were measured using the modified Waldrop scale with fifteen items in six bodily regions; head, eye, ear, mouth, hand, and foot. RESULT: The total scores of Waldrop scale were 4.40+/-1.93(mean+/-standard deviation) in patients and 3.43+/- 1.68 in controls for females, and for males, 4.58+/-1.75 in patients and 4.28+/-1.59 in controls. For females, the excess of MPAs in schizophrenic patients was statistically significant(t-test : p<0.001). For males, schizophrenic patients also showed more MPAs than normal controls, but this tendency did not reach statistical significance (t-test : p=0.094). When the modified Waldrop total scores excluding head circumference were compared, the total scores in schizophrenic patients were significantly higher for both male and female subjects(t-test : male p<0.001, female p=0.001). The individual anomaly items included in Waldrop scale were also investigated. The items of epicanthus, hypertelorism, malformed ears, syndactylia were significantly more frequent in schizophrenic patients. In contrast, the items of adherent ear lobes, asymmetric ears, furrowed tongue, curved fifth finger, single palmar crease and big gap between toes did not show any differences in frequency between schizophrenic patients and normal controls. Since a lot of statistical analyses showed different results between male and female subjects, it seems to be necessary to consider gender as an important controlling variable for the analysis, however only the item of head circumference showed statistically significant gender-related difference according to log-linear analysis. CONCLUSION: With a relatively large sample size, the frequencies of MPAs enlisted in Waldrop scale were compared between schizophrenic patients and normal controls in this study. MPAs were more frequently seen in schizophrenic patients and, especially, several specific items in the Waldrop scale showed prominent excess in schizophrenic patients. Although definite conclusions cannot be drawn due to the inherent limitation of the study using Waldrop scale, these results seem to support the possibility that aberrant neurodevelopmental process might be involved in the pathogenesis of schizophrenia in some of the patients.
Central Nervous System ; Ear ; Ectoderm ; Female ; Fetus ; Fingers ; Foot ; Hand ; Head ; Humans ; Hypertelorism ; Male ; Mouth ; Sample Size ; Schizophrenia* ; Syndactyly ; Toes ; Tongue, Fissured

The Job's syndrome is a relatively rare primary immunodeficiency disorder characterized by recurrent staphylococcal infection and abscess formation, defective neutrophil chemotaxis, and markedly elevated serum IgE level. Clinical features are atopiclike dermatitis, furunculosis, paronychia, pulmonary bacterial pneumonia etc. We reported a case of Job's syndrome in a 12-year-old girl who had recurrent pneumonia, scaly pruritic dermatitis, fissured tongue, and nail dystrophy with elevated serum IgE level.
Abscess ; Chemotaxis ; Child ; Dermatitis ; Female ; Furunculosis ; Humans ; Immunoglobulin E ; Job Syndrome* ; Neutrophils ; Paronychia ; Pneumonia ; Pneumonia, Bacterial ; Staphylococcal Infections ; Tongue, Fissured

Melkersson-Rosenthal syndrome (MRS) is a rare form of hereditary angioedema characterized by a triad of orofacial swelling, relapsing facial paralysis, and a fissured tongue. However, the classic triad is not frequently seen in its complete form, and monosymptomatic or oligosymptomatic forms are more common. Case: A 20-year-old man presented with recurrent labial swelling 9 months ago and recently with a fissured tongue. The surface of the tongue showed deep furrows characteristic of lingua plicata, and an edematous enlargement was observed on the lower lips. Upon neurologic examination, a left sided facial palsy of a peripheral type was noted without abnormalities in the taste sense or lacrimation. An electromyography of the left frontal muscle showed positive sharp waves and fibrillation potentials. Biopsies performed on the lower lips revealed the congested vessels and perivascular inflammatory cells. We report a 20-year-old man with a classical triad of symptoms of Melkersson-Rosenthal syndrome.
Angioedemas, Hereditary ; Biopsy ; Electromyography ; Estrogens, Conjugated (USP) ; Facial Paralysis ; Humans ; Lip ; Melkersson-Rosenthal Syndrome* ; Neurologic Examination ; Tongue ; Tongue, Fissured ; Young Adult