Queilitis granulomatosa, a propósito de dos casos / Granulomatous cheilitis, regarding two cases

La queilitis granulomatosa es una entidad granulomatosa no infecciosa, poco frecuente, que se presenta como un aumento de volumen persistente de la región orofacial. El estudio histológico, junto con la exclusión de otras patologías granulomatosas son necesarios para su diagnóstico, especialmente cuando no se presenta con la triada clásica del Síndrome de Merkelsson Rosenthal. Presentamos dos casos de queilitis granulomatosa y una revisión de la literatura disponible.

Granulomatous cheilitis is a rare, non-infectious, granulomatous entity that presents as a persistent swelling of the orofacial region. Histological study together with the exclusion of other granulomatous diseases are necessary for the diagnosis, especially when the presentation is not the classic triad of Merkelsson Rosenthal Syndrome. We present two cases of granulomatous cheilitis and a review of the available literature.

Research progress on Melkersson-Rosenthal syndrome / 浙江大学学报·医学版

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous syndrome, which is characterized by recurrent orofacial swelling, recurrent facial paralysis and fissured tongue. It has a high prevalence in young adults. Up to now, the etiology of MRS is still not clear, it may related to infection, immune deficiency and hereditary factors. The pharmacological therapy and surgery are the main treatment. Corticosteroids seems to be the drug of choice for MRS patient, but the specific dosage and therapeutic effect have not yet been determined. Surgeries of lips provide excellent results in persistentlip edema MRS cases. This article reviews the research progress on MRS, focusing on its epidemiology, etiology, histopathological characteristics, clinical manifestations, classification, diagnostic criteria, differential diagnosis and treatment, to provide information for its early diagnosis and appropriate treatment.

Melkersson-rosenthal: reporte de caso clínico de una neurodermatosis infrecuente / Melkersson-rosenthal syndrome: case report of an uncommon neurodermatosis

Resumen: El síndrome de Melkersson-Rosenthal (SMR) es una entidad clínica rara, de patogénesis desconocida. Se manifiesta característicamente por edema orofacial recidivante, lengua fisurada y parálisis recurrente del nervio facial. Representando así undesafío diagnóstico y terapéutico, además de generar importante compromiso social al individuo acometido. El presente artículo tiene como objetivo describir el caso de un paciente de 15años de edad que presentó: edema labial, lengua fisurada y queilitis granulomatosa al examen histopatológico, llevándose a consideración la hipótesis del síndrome citado, con resultados satisfactorios al tratamiento establecido.

Abstract: Melkersson-Rosenthal syndrome (MRS) is a rare clinical entity with an unknown pathogenesis. It clinically manifests in orofacial edema, plicated tongue and recurrent paralysis of the facial nerve. It represents a diagnostic and therapeutic challenge, and has an important psycosocial impact on the affected individual. This study describes the case of a 15-year-old patient who presented with labial edema, plicated tongue and granulomatous cheilitis on histopathological examination, for which a diagnosis of MRS was proposed. The patient showed a good response to treatment.

Síndrome de Melkersson-Rosenthal / Melkersson-Rosenthal syndrome

A síndrome de Melkersson-Rosenthal é uma doença rara que pode se apresentar como uma tríade clássica de edema orofacial, paralisia facial e língua fissurada ou, mais frequentemente, com características oligo/monossintomáticas. Relatamos um caso que aportou a um alergista para o diagnóstico de um angioedema, e que na avaliação se configurou como a síndrome completa. Diagnósticos diferenciais com angioedema alérgico, hereditário, idiopático e com outras patologias devem ser considerados. Apesar de o diagnóstico ser clínico, a biópsia cutânea foi relevante. Objetivamos alertar o especialista que se depara com angioedema crônico recorrente sobre essa patologia.

Melkersson-Rosenthal syndrome is a rare disease that may present as the classic triad of orofacial edema, facial paralysis, and fissured tongue or, more often, with oligosymptomatic/ monosymptomatic features. We report a case referred to an allergist for the diagnosis of angioedema that was identified as a complete syndrome during evaluation. Differential diagnoses with allergic, hereditary, and idiopathic angioedema and with other diseases should be considered. Although the diagnosis is clinical, skin biopsy is relevant. Our goal is to alert specialists who deal with chronic recurrent angioedema to the possibility of this syndrome.

Aspectos clínico-patológicos de queilite granulomatosa: relato de caso / Clinical and pathological aspects of Granulomatosis Queilite: Case Report

Introdução:A queilite granulomatosa (QG) é uma lesão oral rara, apresentando clinicamente inchaço de aspecto granulomatoso no lábio.Objetivo:O presente estudo, tem como objetivo apresentar um relato de caso de QG, tendo em vista, a dificuldade do diagnóstico e tratamento de condições inflamatórias com etiologias desconhecidas que acometem a cavidade oral.Relato de caso:Paciente do sexo feminino, apresentou aumento de volume no lábio superior exibindo sintomatologia dolorosa, porém, sem relatar associações com alterações sistêmicas. Durante o exame clínico notou-se que o lábio superior apresentava fissuras profundas, observando tambémedema que se estendia da comissura labial, atravessando a linha média. Levantou-se então, a hipótese diagnóstica de QG. Foi realizada uma biópsia incisional para confirmação do diagnóstico. A análise histopatológica evidenciou, presença de granulomas não caseosos e infiltrado inflamatório linfoplasmocitário perivascular. De acordo com os dados clínicos e análise histopatológica foi dado o diagnóstico inicial de QG. Após o diagnóstico inicial de QG, a paciente foi encaminhada para uma avaliação médica, a fim de avaliar possíveis alterações intestinais que pudessem caracterizar a síndrome de Melkersson-Rosenthal ou Cronh, como também possível reação de hipersensibilidade tardia. Conclusões:O cirurgião-dentista deve estar preparado para diagnosticar aumentos de volume significativos nos lábios, a fim de manejar corretamente o tratamento do paciente, estabelecendo desta forma, prognósticos favoráveis para essas condições (AU).

Introduction:Granulomatous cheilitis (HQ) is a rare oral lesion, presenting clinically granulomatous swelling on the lip. Objective: This study aims to present a case report of HG, considering the difficulty of diagnosis and treatment of inflammatory conditions with unknown etiologies that affect the oral cavity.Objective:This study aims to present a case report of HG, considering the difficulty of diagnosis and treatment of inflammatory conditions with unknown etiologies that affect the oral cavity.Case report:A female patient presented increased volume in the upper lip showing painful symptoms, but without reporting associations with systemic alterations. During the clinical examination it was observed that the upper lip presented deep fissures, also observing edema that extended from the labial commissure, crossing the middle line. The diagnostic hypothesis of GQ was then raised. An incisional biopsy was performed to confirm the diagnosis. Histopathological analysis revealed the presence of non-caseous granulomas and perivascular lymphoplasmacytic inflammatory infiltrate. According to the clinical data and histopathological analysis the initial diagnosis of GH was given. After initial diagnosis of HG, the patient was referred for medical evaluation to evaluate possible intestinal changes that could characterize Melkersson-Rosenthal syndrome or Cronh, as well as possible late hypersensitivity reactions.Conclusions:The dentist should be prepared to diagnose significant volume increases in the lips in order to correctly manage the patient's treatment, thus establishing favorable prognoses for these conditions (AU).

Three Cases of Recurrent Cheilitis Granulomatosa Treated with Variable Combination Therapy / 대한피부과학회지

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.

Síndrome de Melkersson-Rosenthal: Presentación de dos casos pediátricos / Melkersson Rosenthal syndrome: Report of two pediatric cases

El síndrome de Melkersson-Rosenthal es una enfermedad muy poco frecuente en el paciente adulto y excepcional en la niñez y la adolescencia. El reciente trabajo publicado por G. Kayabasoglu et al., afirma que solo 30 pacientes se han descrito en la literatura internacional en edad pediátrica. Es de etiología desconocida, de curso crónico y progresivo, con compromiso neuromucocutáneo que afecta la inervación orofacial con infiltración granulomatosa no caseificante. Clínicamente, se caracteriza por una tríada diagnóstica: edema recurrente de labios y/o cara, parálisis facial periférica aguda recurrente y alternante, y lengua escrotal o geográfica. Existen presentaciones oligosintomáticas y monosintomáticas. Se describen dos pacientes de 7 y 11 años de edad, con presentación completa en un caso y oligosintomática en el otro. Consideramos tener en cuenta esta enfermedad infrecuente como diagnóstico diferencial ante la presencia de parálisis facial periférica aguda recurrente y/o edema de hemicara o labios por su comportamiento evolutivo.

Melkersson Rosenthal syndrome is a very infrequent disease of unknown etiology, chronic and progressive course, with neurocutaneous disease that affects the orofacial innervation and mucocutaneous tissues with non-caseating granulomatous infiltration. Clinically, it is characterized by the diagnostic triad: recurrent edema of lips and/or face; recurrent and alternating acute peripheral facial paralysis and scrotal or geographic tongue. The condition generally presents as oligosymptomatic or monosymptomatic form. Two patients are presented with completed triad and oligosymptomatic form. This recent review published by G. Kayabasoglu et al. states that only 30 patients have been described in the international literature in childhood. So we consider this disease in the differential diagnosis in the presence of acute peripheral facial paralysis and/or lips or facial edema.

Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy

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.

Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy

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.

Cheilitis granulomatosa associated with lupus erythematosus discoid and treated with methotrexate: report of a case

We present the rare case of a 47-year-old patient, suffering from cheilitis granulomatosa and lupus erythematosus discoid: this association is really exceptional because only once reported in English literature. In addition, the treatment of cheilitis granulomatosa is a challenge for the dermatologist: the gold standard, represented by steroids, is in fact designed as a short-time option. Our report confi rms the good efficacy of methotrexate as a steroid-sparing agent.

Orofacial granulomatosis: clinical signs of different pathologies

Orofacial granulomatosis [OFG] is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome

Queilitis granulomatosa de Miescher / Granulomatous Miescher cheilitis

La queilitis granulomatosa de Miescher es la forma monosintomática más frecuente del síndrome de Melkersson-Rosenthal, cuya tríada clásica se completa con la presencia de parálisis facial y lengua fisurada. Es una entidad de causa desconocida, curso crónico eincierto y difícil tratamiento, que muchas veces requiere múltiples modalidades terapéuticas y seguimiento a largo plazo.Se presenta el caso de una paciente con edema labial permanente que evoluciona con múltiples brotes recurrentes...

Queilitis granulomatosa como parte del síndrome de Melkersson Rosenthal / Granulomatous cheilitis as a part of Melkersson Rosenthal syndrome

La queilitis granulomatosa es una entidad infrecuente caracterizada por tumefacción labial recurrente. Es considerada la forma incompleta más frecuente del síndrome de Melkersson Rosenthal, compuesto por la tríada de edema orofacial recurrente, lenguafisurada y parálisis facial. En su histología es característica la presencia de granulomas no caseificantes. Presentamos el caso de una paciente con queilitis granulomatosa y revisamosla bibliografía. Se destaca además esta entidad por su difícil manejo y variables respuestas a los tratamientos instaurados...

Cheilitis Granulomatosa Successfully Treated with Minocycline / 대한피부과학회지

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.

Idiopathic Granulomatous Vulvitis / 대한피부과학회지

Granulomatous vulvitis is a rare localized inflammatory disease characterized by a painless, swollen, indurated and distorted vulva. Histopathologically, it shows chronic non-necrotizing granulomatous inflammation with edema, fibrosis, and lymphangiectasia. Some cases are associated with granulomatous cheilitis or Crohn's disease. So far, only a few cases of idiopathic granulomatous vulvitis have been reported. A 43-year-old female presented with a 1-year-history of persistent swelling of the vulva. The lesion was enlarged, edematous, and indurated, and there were clustered vesicles and papules on the labia major. Histopathologic examination of the swollen labia major showed granulomatous inflammation composed of multinucleated giant cells and epithelioid cells, with numerous lymphocytes in the dermis. Another biopsy specimen obtained from the clustered vesicles showed lymphangiectasia with chronic inflammation. Based on these characteristic histopathologic features and clinical findings, the patient was diagnosed with idiopathic granulomatous vulvitis. Herein, we reported a rare case of idiopathic granulomatous vulvitis that developed in a healthy woman.

Síndrome de Melkersson-Rosenthal: um desafio diagnóstico para o cirurgião-dentista / Melkersson-Rosenthal Syndrome: a diagnosis challenge to the dentist

A síndrome de Melkersson-Rosenthal (SMR) é uma desordem neuromucocutânea rara, de etiologia não conhecida. Ocorre em 0,08 por cento da população. A paralisia facial alternante, queilite grannulomatosa e língua fissurada constituem a clássica tríade de sinal. Paciente de 39 anos do sexo feminino apresentou-se com um quadro de edema em lábio superior e episódios de cefaléia há cerca de 15 anos, com piora lenta e progressiva. Ao exame físico apresentava um edema pronunciado e ressecamento do lábio superior. A língua encontrava-se espessada e com fissuras em toda extensão. O diagnóstico de SMR foi então realizado. O conhecimento das características da SMR é fundamental para o fechamento do quadro. Só desta forma os frequentes erros de dianóstico que são comuns devido ao caráter crônico e insidioso da mesma podem ser evitados.

Queilite granulomatosa associada à síndrome de Melkersson-Rosenthal / Cheilitis granulomatosa associated with melkersson-rosenthal syndrome

A síndrome de Melkerson-Rosenthal (SMR) caracteriza-se por edema orofacial, paralisia facial recorrente e língua plicada. A tríade completa é incomum, com freqüência variando de 8 a 25 por cento, sendo que a apresentação mais comum é a presença de somente um sintoma. A queixa mais freqüente é o edema facial e/ou no lábio. No presente relato, descreve-se o caso de uma jovem, 17 anos, com edema no lábio persistente e língua plicada devido à SMR. A paciente informou que o edema e as alterações na língua haviam se iniciado há 2 anos. Tratamentos prévios haviam sido realizados, porém sem sucesso. Propôs-se a injeção intralesional de 20mg de triancinolona a cada 15 dias, associada a 5mg ao dia de clofazimine por três meses. O lábio voltou ao seu aspecto normal após quatro infiltrações da medicação. Estudos recentes têm considerado a SMR como uma doença granulomatosa, sendo a fase inicial da apresentação orofacial da Doença de Crohn em alguns pacientes. Assim, pacientes com SMR deveriam ser avaliados e seguidos quanto à presença de sintomas gastroenterológicos. O tratamento com corticosteróides tem se mostrado efetivo em reduzir a tumefação do lábio associada a essa doença. Discute-se características clínicas, tratamento e importância da terapia com corticosteróides na paralisia facial associada à SMR.

Melkersson-Rosenthal syndrome (MRS) consists of persistent or recurrent orofacial edema, relapsing facial palsy and fissured tongue. The complete triad of symptoms is uncommon, varying from 8 to 25 percent. The presentation of only one symptom is more common. The most frequent complaint is facial edema and enlargement of the lips. We describe a case of a 17-year-old Brazilian girl with limited edema of the lower lip and fissured tongue due to MRS. Her complaints had started two years before. She referred previous clinical treatments without success. We proposed intralesional injection of triamcinolone at 20 mg every 15 days associated with oral clofazimine at 50 mg/day for three months. The lip became normal after four triamcinolone injections. Recent studies have considered MRS a granulomatous disease, and possibly the initial presentation of CrohnÆs disease in orofacial area of some patients. MRS patients, therefore, should be screened and monitored for gastrointestinal symptoms. Corticosteroid treatment seems to be effective in reducing lip enlargement. We discus the clinical features of this disease, the treatment, and the importance of corticosteroid therapy in cases of MRS-related facial palsy.

Síndrome de Melkerson-Rosenthal: relato de um caso clínico / Melkerson-Rosenthal syndrome: a case report

A Síndrome de Melkersson-Rosenthal (SMR) é caracterizada por uma tríade composta de edema orofacial recorrente, língua fissurada e paralisia facial. No entanto, esta clássica tríade nem sempre é observada, existindo as formas oligossintomáticas e monossintomáticas. Neste trabalho é apresentado um caso clínico de um paciente de 18 anos de idade, com edema labial, língua fissurada e geográfica e eritema gengival, cujas características clínicas e exames de laboratório permitiram o diagnóstico de Síndrome de Melkersson-Rosenthal.

The Melkersson-Rosenthal Syndrome (MRS) is characterized by a triad composed of recurrent orofacial swelling, fissured tongue and facial paralysis. However, this classic triad is not always observed, existing the forms oligossymptomatic and monossymptomatic. In this study is presented a clinical case of a 18 year-old patient, that presented lip swelling, fissured tongue and gingival erythema. Of agreement, with the clinical characteristics and laboratorial analysis the diagnosis was Melkersson-Rosenthal Syndrome.