Traumatic ulcerative granuloma with stromal eosinophilia, an unusual clinical manifestation in the oral mucosa: case report / Granuloma ulcerativo traumático con eosinofilia estromal, una manifestación clínica inusual en la mucosa oral: reporte de un caso

Introduction: Traumatic ulcerative granuloma with stromal eosinophilia is an uncommon condition of the oral mucosa with a chronic course, usually affecting the tongue. Case Report: Clinically it presents as a chronic ulcer, with raised and indurated borders, rarely presented as a tumor. Histologically it shows a diffuse mixed inflammatory infiltrate, rich in eosinophils. The etiology of this lesion is still unclear; however, chronic irritation from traumatic agents is considered a major initiating factor. In some cases, the presence of CD30+ mononuclear cells within the lesions suggest the possibility of a CD30+ lymphoproliferative disorder. This article presents a case of a traumatic ulcerative granuloma with stromal eosinophilia manifested in a 56-year-old female with a solitary ulcerated tumor inside the right cheek. Conclusion: It was diagnosed based on clinical data and histopathological features. In a brief literature review, the entity has been characterized, analyzing its etiology and nature.

Introducción: El granuloma ulcerativo traumático con eosinofilia estromal es una afección infrecuente de la mucosa oral de curso crónico, que suele afectar a la lengua. Case Report: Clínicamente se presenta como una úlcera crónica, con bordes elevados e indurados, rara vez se presenta como un tumor. Histológicamente muestra un infiltrado inflamatorio mixto difuso, rico en eosinófilos. La etiología de esta lesión aún no está clara; sin embargo, la irritación crónica por agentes traumáticos se considera un factor de iniciación importante. En algunos casos, la presencia de células mononucleares CD30 + dentro de las lesiones sugiere la posibilidad de un trastorno linfoproliferativo CD30+. En este artículo se presenta el caso de un granuloma ulcerativo traumático con eosinofilia estromal que se manifiesta en una mujer de 56 años con un tumor ulcerado solitario en el interior de la mejilla derecha. Conclusión: Se diagnosticó con base en datos clínicos y características histopatológicas. En una breve revisión de la literatura se ha caracterizado la entidad, analizando su etiología y naturaleza.

Granuloma faciale: a rare disease from a dermoscopy perspective / Granuloma facial: uma doenca rara sob uma perspectiva dermatoscopica

The granuloma faciale is a rare and benign skin disease of unknown etiology, characterized by chronic leukocitoclastic vasculitis. It is characterized by skin lesions predominantly facial whose course is chronic and slowly progressive. The diagnosis is based on clinical features, histopathology and, more recently, in dermoscopy. We describe the case of a male patient, 40 years old, with a sarcoid lesion on the malar site, whose histopathological examination revealed a mixed inflammatory infiltrate with presence of Grenz zone. Dermoscopy revealed a pink background with white striations. The definitive diagnosis is made by histopathologic evaluation, and dermatoscopy can be helpful. It is known to be resistant to therapy, oral medications, intralesional and surgical procedures are options.

O granuloma facial é doença cutânea rara e benigna, de etiologia desconhecida, caracterizado por vasculite leucocitoclástica crônica. Caracteriza-se por lesões cutâneas predominantemente faciais, tem curso crônico e lentamente progressivo. O diagnóstico é baseado na clínica, histopatologia e, mais recentemente, na dermatoscopia. Relatamos o quadro de um paciente masculino, 40 anos de idade, com lesão sarcoídea na face malar, cujo exame histopatológico revelou infiltrado inflamatório misto com presença de zona de Grenz. A dermatoscopia revelou um fundo rosado com estrias brancas. O diagnóstico definitivo é feito pela avaliação histopatológica, sendo que a dermatoscopia pode ser útil. É conhecida por ser resistente à terapêutica, sendo propostas medicações orais, intralesionais e procedimentos cirúrgicos.

Granuloma eosinófilo craneal solitario: caso clínico / Solitary eosinophilic granuloma of the skull: a clinic case

La histiocitosis de las células de Langerhan (HCL), es una enfermedad Granulomatosa de etiología no bien precisada todavía, la cual puede tener diferentes manifestaciones y localizaciones,1 aunque algunos la consideran una proliferación neoplásica de las células de Langerhan que aparecen en el escenario de sitios nodulares o no.2 La Histiocytosis X incluye tres componentes: El Granuloma Eosinófilo, la enfermedad de Hand-Schüller-Christian y el síndrome de Letterer-Siwe.5 El Granuloma Eosinófilo es un tumor óseo benigno muy raro, que se presenta en más del 90 por ciento de los casos en niños menores de 10 años, 6 teniendo además cierta predilección por el sexo masculino. Esta lesión es usualmente encontrada en los huesos largos y planos. Se trata de una paciente femenina de 50 años de edad con antecedentes de procesos alérgicos y asma bronquial grado II y fumadora inveterada, que hacía 8 meses, previo a su ingreso, había comenzado a presentar cefalea holocraneal, fue notando irregularidades en su cuero cabelludo y fue hospitalizada porque al realizarle una radiografía simple de cráneo se evidenció una imagen radiotransparente de bordes mal definidos, lo que fue constatado por tomografía simple de cráneo, la paciente fue operada, realizándole una craniectomía y excéresis de un tejido Granulomatoso sobre la duramadre con colocación de injerto de cemento quirúrgico en el área de la craniectomía. Se le diagnosticó un Granuloma Eosinófilo. Posterior a su recuperación fue tratada con oncología donde se le aplicó radioterapia. La evolución de la paciente fue satisfactoria, ahora se encuentra asintomática.

The Langerhans cells hystiocitosis is a Granulomatous ill, it has unknown etiology today. This disease can be many places and clinical manifestations, although some authors consider it a neoplasic proliferation of the Langerhans cells which can be on nodular region scenery or not. X Hystiocitosis include three diseases: Eosinophilic Granuloma, Hand-Schuller- Chritian disease and Letterer-Siwe syndrome. Eosinophilic Granuloma is a very uncommon benign bone tumor, which may be present in more than 90 percent in childhood under 10 years old. They have predominance on male patients. Usually it is found on long and flat bones. The case report is about 50 years old, female patient, with allergic history and bronchial asthma II degree, she come to the hospital because she don’t filled well since 8 months ago and she complained mild headache, furthermore she noticed irregularity over her skull, on vertex region, at her admission she was screening with cranial X ray, where was found a radio lucid images over the painful area, moreover it had irregular edge whit more than 10 cm long. After that she was screening on CT-scan, where was found a very large osteolytic lesion without intracranial grow. She was approached with craniectomy and graft of surgical cement. She was diagnosed Eosinophilic Granuloma on pathologic department. After her discharge she was treated by oncology, here she received holocraneal radiotherapy. Her outcome has been satisfactory because she is asymptomatic and her CT-scan showed not evidences of recidivism.

Granuloma eosinofílico da coluna vertebral cervical em criança / Eosinophilic granuloma of the cervical spine in children

Granuloma eosinofílico (GE) refere-se à forma benigna e mais frequente de histiocitose de células de Langerhans. Trata-se de uma doença que acomete principalmente crianças e adolescentes, sendo rara a sua ocorrência em adultos. Constitui a forma localizada de proliferação de histiócitos em crânio e ossos longos. O acometimento vertebral é incomum, sendo a localização cervical a menos descrita. Neste artigo, é descrito um caso de uma criança de 7 anos de idade com GE na coluna vertebral cervical com compressão medular. A criança evoluiu com regressão dos sintomas após tratamento conservador, tendo permanecido assintomática ao longo de seguimento de dois anos.

Eosinophilic granuloma (EG) is a benign and more frequent form of Langerhans cell histiocytosis. Children and adolescent are mainly affected, being rare in adults. There is local proliferation of histiocytes in skull and long bones. Vertebral involvement is uncommon, especially at the cervical local. In this paper, we describe a case of a 7 years old child with EG in the cervical spine with spinal cord compression. The patient presented with regression of symptoms after conservative treatment and remained asymptomatic at the two years follow-up.

Uso de corticoide intralesional en histiocitosis de langerhans crónica focal: reporte de un caso / Use of intralesional corticoids in focal chronic langerhans cell histiocytosis: A case report

El término Histiocitosis de células de Langerhans (HCL) comprende a un grupo de enfermedades que son el resultado de una alteración a nivel del sistema fagocítico mononuclear. En función de los hallazgos clínicos la HCL, se clasifican en tres grupos por fines pronósticos y terapéuticos: 1.- Crónica Focal, por lo general lesión solitaria de un hueso, sin afectación de otros órganos ni partes blandas (ex Granuloma Eosinófilo); 2.- Crónica Diseminada, (ex Hand-Schuller-Christian); 3.- Aguda Diseminada (ex Letterer-Siwe). La etiología de este grupo de lesiones permanece incierta, aunque se han propuesto: infección, inflamación y reacciones inmunológicas. Presentamos el caso clínico de un paciente de género femenino, de 12 años de edad, que consulta por aumento de volumen en la zona del ángulo mandibular derecho con un mes de evolución, no doloroso. Se realiza ortopantomografía y TAC, observando destrucción de cortical vestibular y lingual. Se realiza la biopsia, se somete a estudio inmunohistoquímico, dando el diagnóstico de HCL Crónica Focal, fue tratado con Triancinolona de 40 mg (Kenalog - 40) en 2 dosis, con control de 3 años y sin recidivas a la fecha.

The term Histiocitosis of Langerhans's cells (LCH) covers a group of diseases that are the result of an alteration to the mononuclear phagocyte system. According to the clinical findings, the HCL is classified in three groups for predictable and therapeutic purposes: 1.- Focal Chronic, in general a lone injury of a bone, without affecting other organs or soft parts (Ex-Eosinophilic granuloma); 2.-Spread Chronic, (ex-Hand-Schuller-Christian); 3.- Spread Acute (ex-Letterer-Siwe).The etiology of this group of injuries remains unknown, eventhough it has been proposed: infection, inflammation and immunological reactions. Here, we present a clinical case: A 12-year-old female patient that consults for increase of volume in mandibular right back area, with a one month of evolution, not painful. Panoramic radiograph and TAC images are showing, observing destruction of vestibular and lingually bone. The biopsy was submitted to immunohystochemical study, giving the diagnosis of Chronic Focal LCH, it was treated with Triancinolona 40 mg (Kenalog - 40) in 2 doses, after 3 years of following up there is not signs of recurrence at the date.

Granuloma de células de Langerhans intraorbitário extraocular: abordagem cirúrgica primária. Por quê? - Relato de caso / Intraorbital extraocular Langerhans' cell granuloma: primary surgical approach. Why? - Case report

Apresentamos um caso no qual foi realizada orbitotomia de urgência em paciente com granuloma de células de Langerhans intraorbitário o qual apresentava comprometimento anatômico e funcional da função visual devido à hipertensão intraorbitária. Paciente masculino, 2 anos, apresentou exoftalmia, proptose conjuntival, olho vermelho, oftalmoplegia completa com midríase não fotorreativa e ptose palpebral direita. À fundoscopia apresentava engurgitamento venoso com edema de papila à direita. Foi realizada abordagem cirúrgica com orbitotomia via intracraniana sendo removida a lesão com remissão total da clínica tanto anatômica quanto funcionalmente. O manejo do granuloma de células de Langerhans pode variar desde a abordagem cirúrgica primária, passando pela terapia com esteróides até quimioterapia e radioterapia. No caso, a cirurgia foi a primeira opção devido ao e iminente risco de perda da função visual.

We present a case in which a patient with intraorbital Langerhans' cell granuloma, with anatomical and functional skills compromised by intraorbital hypertension, which was treated with urgency orbitotomy by cranial approach. Male, two years-old, presented conjuntival proptosis, red eye, complete ophthalmoplegy, exophthalmos, with non-fotoreactive mydriasis and blepharoptosis of the right eye. Fundoscopy presented venous enlargement with optic disc edema. A cranial approach with orbitotomy was carried out, removed the lesion with total remission of clinical presentation and anatomic appearance as well. The handling of Langerhans' cell granuloma can vary from primary surgical approach as corticotherapy to chemotherapy and radiotherapy. In this case, the surgery was the first option due to the imminent risk of loss of visual function.

Granuloma eosinofílico orbitário / Orbital eosinophilic granuloma

OBJETIVO: Relatar, de forma retrospectiva, a experiência no diagnóstico e tratamento do granuloma eosinofílico da órbita em serviço de referência. MÉTODOS: Identificados os pacientes com diagnóstico de granuloma eosinofílico orbitário, no Registro do Laboratório de Patologia Ocular do Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, no período entre 1974 e 2004. Coletados dados referentes ao diagnóstico, tratamento e realizada revisão das lâminas coradas pela hematoxilina e eosina. RESULTADOS: Foram identificados 3 casos com idade de 1, 6 e 11 anos. Em nenhum caso o diagnóstico de granuloma eosinofílico foi aventado pré-operatoriamente. Todos pacientes foram submetidos a biópsia, exibindo aspecto anatomopatológico típico. Em um dos casos, após a biópsia, havia tomografia computadorizada da órbita disponível, exibindo padrão característico. Todos os pacientes foram tratados com corticoesteróides sistêmicos, com resolução do tumor orbitário. CONCLUSÃO: Trata-se de doença rara, de difícil diagnóstico clínico sem avaliação radiológica. Apesar da grande destruição tecidual, apresenta boa resposta ao tratamento com corticoesteróides.

PURPOSE:To report the retrospective experience of a reference center in the diagnosis and treatment of orbital eosinophilic granuloma. METHODS: A review of the files in the Opththalmic Pathology Laboratory, of the Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, between 1974 and 2004, was conducted to identify cases of orbital eosinophilic granuloma. Data of diagnosis and treatment were collected. The hematoxilin and eosin stained sections were reviewed. RESULTS: Three cases with ages of 1, 6 and 11 years were retrieved. In none of them the clinical diagnosis of eosinophilic granuloma was suspected. All patients underwent diagnostic biopsy with characteristic histological aspect. In one case a orbital computed tomography after biopsy exhibited typical findings. All patients improved with systemic steroids./ CONCLUSIONS: This is a rare disease, with a difficult clinical diagnosis if radiological evaluation is not available. In spite of its aggressiveness at presentation, the disease shows good response to systemic steroids.

Remisión espontánea de histiocitosis de células de Langerhans unifocal de la mandíbula / Spontaneous Remission of Langerhans cell histiocytosis of the mandible unifocal

La histiocitosis de células de Langerhans, denominada originalmente histiocitosis X, comprende un grupo de desórdenes caracterizados por la proliferación anormal de este tipo de células acompañadas con abundante cantidad de esosinófilos. La enfermedad puede afectar cualquier estructura u órgano del cuerpo ya sea en forma localizada denominada granuloma eosinófilo o en formas sistémicamente diseminadas que dependiendo de la magnitud de las lesiones y edad del paciente reciben el nombre de enfermedad de Hand- Schuller-Christian o Letterer-Siwe. En este trabajo se presenta un caso de histiocitosis de células de Langerhans unifocal en una niña de 8 años de edad que había producido fractura patológica a nivel del cuerpo mandibular. La lesión se resolvió espontáneamente después de haber tomado una biopsia para diagnóstico y haber fijada la fractura. No se realizó ningún tratamiento adicional. Se presentan las características clínicas y radiográficas iniciales, la histología e inmunopatología de diagnóstico y los hallazgos a los 190 días de control.

Langerhans'cell histiocytosis, formely known as histiocytosis X, comprises a group of disorders that implies an abnormal proliferation of these kind of cells and usually a massive aggregationof eosinophils. It affects any site and organ of the body, either as an isolated lesion, named eosinophilic granuloma, which is a localized form, or as a widespread systemic disease that correspond to Hand- Schuller-Christian and Letterer-Siwe diseases. We report a case of Langerhans-cell histiocytosis localized in the mandible of an 8-year-old girl which produced fracture of this bone. The lesion resolved spontaneously after a diagnostic biopsy and stabilization of the fracture No additional treatment was provided. The initial clinical and radiographic features, the histopathology and immunopatholy of the lesion and the evolution control after 190 days follow up of the case are presented.

Granuloma eosinofílico de ganglio linfático. Presentación de un caso / Eosinophilic granuloma of lymphatic ganglion. Case presentation

Se presenta un paciente de un año y medio de edad con adenopatías cervicales correspondientes a un granuloma eosinofílico. El estudio radiológico del esqueleto y la evolución confirmaron la localización ganglionar primaria de esta variante de granulomatosis de células de Langerhans (histiocitosis X).

A patient of one year old an a half presented with cervical adenopathies corresponding to an eosinophilic granuloma. Radiologiocal study of the spine and its evolution confirmed the primary ganglionic localization of this variant of Langerhands' cells granulomatosis (histiocytosis X).