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.

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.

Synchronous Orofacial Granulomatosis in a Patient with Brain Cavernous Hemangioma

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.

Manifestations oto-rhino-laryngologiques des maladies systémiques

Les atteintes oto-rhino-laryngologiques sont parmi les plus fréquentes des manifestations observées dans les maladies systémiques et rhumatologiques. Une grande variété de pathologies a été décrite telles que la polychondrite atrophiante, certaines granulomatoses et connectivites ou la sarcoïdose. Dans d'autres cas, l'atteinte ORL est plus rare mais peut, parfois, révéler la maladie et poser des difficultés diagnostiques. Une étroite collaboration entre spécialistes ORL, rhumatologues et internistes est indispensable pour améliorer l'approche diagnostique et thérapeutique chez ces patients

Orofacial granulomatosis and diet therapy: a review of the literature and two clinical cases

Abstract: Orofacial granulomatosis is a nonspecific term that contains a wide variety of granulomatous entities, which share a clinical and histopathological presentation. It manifests as persistent or recurrent orofacial swelling, amongst other findings. Idiopathic orofacial granulomatosis, characterized by an absence of systemic granulomatous disease, is a diagnosis of exclusion. The main differential diagnosis is Crohn's disease. Its pathogenesis is unknown, however, it seems to be immune-mediated. Patch-test sensitivity to multiple allergens is well documented. Currently, therapeutic options consider restrictive diets, topical, intralesional, and systemic agents. First-line therapy is currently a matter of debate. We present a review of the value of diet therapy in this syndrome, along with two illustrative cases.

Leishmaniasis cutáneo-mucosa: presentación de un caso con compromiso medio-facial / Mucocutaneous leishmaniasis: report of a case with midd lefacial commitment / Lehismaniasis mucocutânea: apresentação de um caso com o compromisso mid-facial

La leishmaniasis es una zoonosis parasitaria causada por protozoos. Puede afectar la piel y las mucosas o presentarse como una enfermedad visceral. La variedad mucocutánea conduce a la destrucción parcial o completa de las membranas mucosas de la nariz, las fauces y la faringe. Aproximadamente un 90% de los casos con afectación mucocutánea se producen en Brasil, Bolivia y Perú. En nuestro país afecta en forma endémica a las provincias del norte desde principios del siglo XX. Se relata el caso de un paciente de 53 años con odinodisfagia de aproximadamente 6 meses de evolución, asociado a formaciones granulomatosas medio- faciales, en el que se diagnosticó leishmaniasis cutaneomucosa mediante el rescate de amastigotes en muestras tomadas de lesiones de paladar blando para estudio anatomopatológico con tinción de Giemsa. Se realizó tratamiento con meglumina antimoniato con buena evolución clínica a partir de los quince días de instaurado el mismo.

Leishmaniasis is a parasitic zoonosis caused by protozoa. It can affect skin, mucous membranes or presented as visceral disease. Mucocutaneous variety leads to partial or complete destruction of the mucous membranes of the nose, mouth and pharynx. Approximately, 90% of cases with mucocutaneous involvement occurs in Brazil, Bolivia and Peru. In our country it affects endemic to the northern provinces since the beginning of the century. The case of a 53-year-old patient with odinodisphagia of approximately 6 months of evolution, associated with mid-facial granulomatous formations in which cutaneomucous leishmaniasis was diagnosed by rescue of amastigotes in samples taken from lesions of soft palate for anatomopathological study with Staining of Giemsa. Treatment with meglumina antimonia was carried out with good clinical evolution from the fifteen days of the same establishment.

A leishmaniose é uma zoonose parasitária causada por protozoários. Ele pode afectar a pele e membranas mucosas ou presente como doença visceral. variedade mucocutânea conduz à destruição parcial ou completa das membranas mucosas do nariz, boca e faringe. Aproximadamente 90% dos casos com envolvimento mucocutânea ocorrem no Brasil, Bolívia e Peru. Em nosso país que afeta endêmica para as províncias do norte, desde o início do século XX. O caso de um odinodisfagia 53 anos, aproximadamente, 6 meses evolução associada com formações granulomatosas mediofaciais em que a leishmaniose mucocutânea foi diagnosticada por resgatar amastigotas em amostras tomadas a partir de lesões do palato mole para estudo histopatológico contou Giemsa. O tratamento foi realizado com antimoniato de meglumina com boa evolução clínica a partir de quinze dias introduzidas ele.

Foreign Body in Jugal Mucosa

Introduction Foreign body in the oral cavity may be asymptomatic for long time and only sometimes it can lead to a typical granulomatous foreign body reaction. Some patients may complain of oral pain and present signs of inflammation with purulent discharge. A granuloma is a distinct, compact microscopic structure composed of epithelioid-shaped macrophages typically surrounded by a rim of lymphocytes and filled with fibroblasts and collagen. Nowadays, the increase of cosmetic invasive procedures such as injection of prosthetic materials in lips and cheeks may lead to unusual forms of inflammatory granulomas. Objectives Describe an unusual presentation of a foreign body reaction in the buccal mucosa due to previous injection of cosmetic agent. Resumed Report A 74-year-old woman was referred to the Department of Otorhinolaryngology, Head and Neck Surgery to investigate the presence of multiple painless, bilateral nodules in the buccal mucosa, with progressive growth observed during the previous 2months. The histologic results showed a foreign body inflammatory reaction. Conclusion Oral granulomatosis lesions represent a challenging diagnosis for clinicians and a biopsy may be necessary. Patients may feel ashamed to report previous aesthetic procedures, and the clinicians must have a proactive approach.(AU)

Manifestaciones otorrinolaringológicas de la granulomatosis con poliangitis (Wegener) / Otolaryngological manifestations of granulomatosis with polyangitis (Wegener's)

La Granulomatosis con Poliangitis (GPA) se caracteriza por una vasculitis granulomatosa de vías aéreas y glomerulonefritis. Han surgido múltiples avances en su diagnóstico y tratamiento, sin embargo su etiología permanece sin aclarar. Objetivos: Esta revisión guía a los otorrinolaringólogos a las manifestaciones clínicas principalmente las que se ubican en la región de cabeza y cuello, formas de diagnóstico y bases de tratamiento, las cuales deben realizarse de forma precoz para mejorar el pronóstico, evitar la progresión y disminuir las complicaciones. Metodología: Revisión de la literatura a través de las bases de datos Scielo, Science Direct y Pubmed, entre los años 1993 y 2014. Se incluyeron revisiones, artículos, reportes de casos clínicos, tratados de Otorrinolaringología. Resultados: Las alteraciones más frecuentemente identificadas en la GPA son: obstrucción nasal, costras nasales, rinorrea, otalgia, tinnitus, otitis media de difícil manejo, disfonía y disnea. Conclusiones: Las manifestaciones de la Granulomatosis con Poliangitis son múltiples y frecuentes en la región de la cabeza y cuello, su sospecha como diagnóstico diferencial en pacientes con presentaciones poco comunes y de difícil manejo o evolución tórpida siempre debe estar presente...

Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis, is characterized by granulomatous vasculitis of the respiratory tract and glomerulonephritis. There have been many advances directed towards its diagnosis and treatment, even though its etiology remains unclear. Objectives: This review summarizes the clinical manifestations of the disease, focused on the ones located in the head and neck region. GPA´s diagnosis and treatment, which must be done as early as possible in order to improve the prognosis. Methods: Review of the literature by searching SciELO, PubMed and ScienceDirect databases between 1993 and 2014. Reviews, original research articles, case reports and otolaryngology treaties were included. Results: GPA’s frequent alterations are: nasal scabs and obstruction, rhinorrhea, earache, tinnitus, otitis media unwieldy, dysphonia and dyspnea. Conclusions: GPA’s clinical manifestations are usually multiple and very common in the head and neck. GPA has to be kept in mind as differential diagnosis in patients with unusual presentations and those who are difficult to manage...

Orofacial Granulomatosis Associated with Crohn's Disease

Orofacial granulomatosis (OFG) is a term used to describe swelling of the orofacial area, mainly in the lips, secondary to an underlying granulomatous inflammatory process. OFG has been reported in association with systemic conditions such as sarcoidosis and Crohn's disease (CD). OFG may precede gastrointestinal disease, such as CD, by several years and may be the only obvious focus of the disease. Herein, we report a patient with OFG and non-symptomatic ulcerations of the ileocecal valve. The patient received intralesional triamcinolone injections every 2 weeks. After 6 weeks, all oral lesions showed marked improvement. The favourable treatment response of this patient suggests that intralesional triamcinolone can be used as a treatment option for patients with CD that have oral lesions. In addition, patients presenting with OFG should be carefully evaluated for gastrointestinal signs and symptoms.

Manifestações otorrinolaringológicas mais comuns das doenças granulomatosas / The most common otorhinolaryngologic manifestations of granulomatous diseases

Introdução: As doenças granulomatosas são decorrentes de processos imunopatológicos em que há falha na fagocitose de organismos intracelulares. Podem ocasionar úlceras nas mucosas orais, nasais e faríngeas, lesões nas pregas vocais, otorréia e lesões vegetantes orofaríngeas. Objetivo: Descrever as manifestações otorrinolaringológicas mais freqüentes em doenças granulomatosas comuns: hanseníase, paracoccidioidomicose e leishmaniose. Método: Realizou-se estudo retrospectivo dos prontuários de pacientes diagnosticados com as doenças citadas acima, do período de 1º de janeiro de 2005 a 31 de outubro de 2007, no ambulatório de infectologia de um hospital terciário. Resultados: Avaliaram-se 142 pacientes; 93 com diagnóstico de leishmaniose, 39 de paracoccidiodomicose e 10 de hanseníase. Dos 93 casos de leishmaniose, 16 (17,2%) apresentavam comprometimento mucoso, sendo os sinais mais freqüentes a perfuração septal e úlcera em mucosa nasal, ambos com 8 casos. Na paracoccidiodomicose, a lesão mucosa mais freqüente foi a ulceração orofaríngea com 15 casos (38,4%). Conclusão: Sinais e sintomas de cabeça e pescoço são comuns nos pacientes com leishmaniose e paracoccidioidomicose. As manifestações nasais prevalecem na leishmaniose e as orofaríngeas na paracoccidioidomicose.

Introduction: Granulomatous diseases result from immunopathologic processes in which there is a failure in the fagocitosis of intracellular organisms. They can cause oral, nasal and pharyngeal mucosa ulcers, vocal cords lesions, otorrhoea and oropharyngeal vegetant lesions. Objective: Describing the most frequent otorhinolaryngologic manifestations in common granulomatous diseases: hanseniasis, paracoccidioidomycosis, leishmaniasis. Method: A retrospective study has been carried out from records of patients diagnosed with the abovementioned diseases between January 1, 2005 and October 31, 2007 in an infectology ambulatory of a tertiary hospital. Results: 142 patients were evaluated; 93 with leishmaniasis, 39 with paracoccidioidomycosis and 10 with hanseniasis. In 93 cases of leishmaniasis, 16 (17.2%) had mucosal affection, and the most common signs were septum perforation and nasal mucosal ulcers, both with 8 cases. In paracoccidioidomycosis, oropharyngeal ulcer was the most frequent, with 15 cases (38,4%). Conclusion: Head and neck signs and symptoms are common in patients with leishmaniasis and paracoccidioidomycosis. Nasal manifestations prevail in leishmaniasis and oropharyngeal ones in paracoccidioidomycosis.

Nasal septum perforation: rare manifestation of Crohn's disease.

We report a 25-year-old man with four-year history of weight loss, diarrhea and intermittent epistaxis. Endoscopy and biopsy showed inflammation of the nasal mucosa. Endoscopic and histologic diagnosis of Crohn s disease was made later; the granulomatous nasal lesions responded to sulfasalazine and azathioprine.

Granuloma faciale: an unusual presentation

Granuloma faciale is a rare skin disorder, characterized by asymptomatic cutaneous nodules on the face without any systemic manifestations. The disorder mainly affects middle-aged Caucasian males. One such case occurring in a young unmarried lady of 'Pathan' descent is presented here. The presentation was unusual due to the history of frequent swelling, redness and heat in her nodules without any known stimuli and spontaneous regression of the nodules to the original size in a few hours. The patient was treated with a combination of cryotherapy and intralesional corticosteroids with excellent results