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.

Quiste óseo aneurismático simulando tumoración de rinofaringe / Aneurismatic bone cyst simulate rhino pharyns tumor

Los quistes óseos aneurismáticos del área de cabeza y cuello representan menos del 5% de todos los tumores óseos. Son lesiones benignas, que comúnmente afectan las metáfisis de los huesos largos y las vértebras como en el caso que se describe a continuación, simulando una lesión de la rinofaringe. Se describe su manejo y tratamiento.

The aneurismal of bone cyst of the head and neck localization, represent less than the 5% of the all bone tumors. They are benign lesions, and commonly affecting the metaphysis of the long bones and vertebrae, like in the case we described below, it simulating the rhino pharynx lesion. We describe the management and treatment.

A case report of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma.

A case of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma in a child is reported where the frontal, zygomatic and maxillary bones of the left orbital wall are affected. As eosinophilic granuloma is a rare disease and the involvement of maxillary bone in orbital eosinophilic granuloma is not well documented in the literature, this case is reported for its unusual involvement of 3 bones at a single site. The diagnosis was established by clinical, radiological and histological findings. As no other system or site was involved and considering the osseous involvement of multiple bones at one site, local radiotherapy was preferred as the mode of treatment. The patient responded favourably to radiotherapy with reduction of proptosis and tumour mass but there was no visual recovery. In the absence of universal agreement over the mode of treatment, the result emphasises the probable benign nature of the tumour and the need to withhold more aggressive treatment modalities for extensive multisystem involvements.

Kimura's disease--cytodiagnostic pointers: a case report.

Kimura's disease is an entity which is common in Asia with predilection for males. Earliest reports were from China. Kimura et al reported the case in detailfrom Japan 1948. Cytological diagnosis of Kimura's helps in allaying fears of malignancies in clinicians and patients. When all the cytological features are seen, this diagnosis should be borne in mind, and the differential diagnosis should be ruled out. Here we report 2 cases of Kimura's disease diagnosed on cytology and confirmed on biopsy. The etiology of Kimura's is unknown but a hypersensitivity etiology has been proposed.

Langerhans cell histiocytosis of the mandible in a six-year-old child.

Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis--solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.

Eosinophilic granuloma: a rare presentation of Langerhans cell histiocytosis in a young female

Histiocytosis encompasses a group of diverse disorders that have in common, as a primary event, the accumulation and infiltration of monocytes, maerophages, and dendritic cells in the affected tissues. Langerhans cell histiocytosis [LCH] can be local and asymptomatie, as in isolated bone lesions, or it can involve multiple organs and systems with significant symptomatology and consequences. Thus, the clinical manifestations depend on the site of the lesions and organs and systems involved and theft functions. We report a case of a 35-year-old lady who presented with skin lesions in presternal area mimicking very closely to those of scrofuloderma [a form of skin tuberculosis]. She had been treated with antituberculous therapy thrice with no benefit. There was no evidence of systemic involvement. Diagnosis of LCH was confirmed on histopathological findings of skin lesion and markers. Patient was treated with oral methttrexate with resolution of lesions

Granuloma eosinofílico primário do pulmão: registros clássicos / Primary eosinophilic granuloma of the lung: classical records

Foram analisados, retrospectivamente, cinco casos de granuloma eosinofílico primário do pulmão (GEPP) identificados durante admissão no Hospital Universitário Lauro Wanderley da Universidade Federal da Paraíba (HULW/UFPB), no interregno de 1982 a 1998, admitidos com história diagnóstica de fibrose intersticial difusa (FID). Foram revisados os seguintes aspectos: formas de apresentação clínica e epidemiológica, presunção diagnóstica na admissão, doenças concomitantes e evolução terapêutica. A presunção clínica do diagnóstico foi confirmada mediante a citometria com níveis elevados de CD1 no lavado broncoalveolar (LBA). A biópsia pulmonar aberta foi indicada pelos sintomas persistentes em acompanhamentos ambulatoriais sem melhora clínica. O relatório anatomopatológicodemonstrou histiocitose de células de Langhans. A terapia à base de corticosteróides não apresentou boa eficácia, uma vez que, observando a evolução clínica dos casos apresentados, ocorreram dois óbitos e os demais não tiveram uma melhora significativa da enfermidade de base, porém existiu um prejuízo nessa avaliação, em virtude do não-abandono do vício tabágico. Enfatiza-se a fisiopatogenia e a importância dessa enfermidade, apesar de rara e pouco conhecida, como causa de FID, ilustrando um dos casos que se reveloubastante exuberante no diagnóstico de GEPP.

We studied retrospectively five cases of eosinophylic granuloma of the lung identified during admission at HULW/UFPB from 1982 to 1998. They were admitted with diagnostical history of difuse intersticial fibrosis. The following aspects were reviewed: clinical and epidemilogical presentation, diagnostic presumption at admission, concomitant diseases and evolution. The diagnostic was confirmed with bronchoalveolar lavage (BAL) and lung biopsy. The anatomicopathologic exams report histiocytosis of Langhans'cells. The clinical course was variable. There are no accepted successful treatment, although three patients responded poorly to corticosteroids and twodied, in spite of all effort in the treatment with corticosteroids too.

Eosinófilos: su rol en la patología dermatológica severa: primera parte / Eosinophils: the role in severe dermatologic disease: first part

El eosinófilo es un granulocito pequeño derivado de la médula ósea, tiene un núcleo bilobulado característico y gránulos citoplasmáticos responsables de muchas de sus funciones pro inflamatorias; estas células interactúan con otras por la expresión de múltiples receptores en su superficie, lo que la convierte en una célula efectora de la respuesta inmune con importante tropismos hacia los tejidos. La producción y acumulación de eosinófilos implica la proliferación y diferenciación de células hematopoyéticas con las células endoteliales, la quimiotaxis, activación celular y el balance entre la sobrevida y apoptosis del eosinófilo. La eosinofilia persistente en sangre periférica puede ocurrir en un gran número de enfermedades y en algunas el eosinófilo es la principal célula efectora. La eosinofilia ocurre en una variedad de enfermedades severas en las que se destacan las enfermedades alérgicas, infecciones parasitarias y neoplasias. Las enfermedades cutáneas en las que existe una infiltración tisular por eosinófilos constituye un grupo aparentemente heterogéneo, que se ha denominado dermatosis eosinofílica. Existe un grupo de desórdenes donde no se conoce el origen de la eosinofilia que se ha denominado "síndrome hipereosinofílico", en el cual la piel también es un órgano blanco de estas células. Es importante conocer la función efectora del eosinófilo en las reacciones inflamatorias cutáneas y de otros órganos, su papel modulador y las circunstancias donde predominan sus efectos citotóxicos generadores de enfermedad

Tumores pared torácica / Thoracic wall tumours

Los tumores de la pared torácica son entidades raras de encontrar, entre las que seincluyen lesiones de hueso y partes blandas, pueden ser primarias o secundarias (metástasis, infecciosas) incluyendo también tumores de estructuras adyacentes al tórax que comprometen la pared torácica como mama, pulmón, pleura y mediastino. Se presentan dos casos clínicos, que consultan por dolor costal, diagnosticándose en ambos un tumor costal, tratados con exéresis costal. La evolución postoperatoria fue satisfactoria y las biopsias demostraron una Displasia Osea Fibrosa y con Granuloma Eosinofilo.

Granuloma eosinofilo solitario de vertice de orbita y senos paranasales en un adulto: comunicacion de un caso y revision de la literatura / Adult solitary eosinophilic granuloma of the orbital vertex and paranasal sinuses: case report and review

Objective: to report a rare localization of Langerhans cell histiocytosis and to define its differential diagnosis and therapeutic options. Description: a 32 year-old male with derecasing visual acuity, headache and epistaxis. MRI: parasellar lytic lesion extending to nasal cavities. Transnasal biopsy: proliferation of S100 positive cells and eosinophilic granulocytes. Intervention: radiation therapy was followed by reission. Conclusion: Langerhans cell histiocytosis can present as a unifocal (eosinophilic granuloma) or multifocal sidorder. Usually affects children. The skull base is rarely affected. Surgery with or without radiotherapy is the treatment of choice for solitary accessible lesions. Isolated radiotherapy and intralesional steroids are valid options. Systemic disease requires chemoterapy

Biopsias guiadas por marcación radioisotópica para alteraciones costales solitarias presuntivamente neoplásicas / Radioisotope-guided rib biopsies in suspected neoplastic patients

Antecedentes: El centellograma óseo se usa rutinariamente para estadificación y seguimiento de tumores malignos, especialmente de aquellos que con frecuencia dan origen a metástasis esqueléticas. Anormalidades costales múltiples alejadas de las articulaciones, son generalmente compatibles con metástasis. Pero por otro lado, un sitio solitario de hipercaptación puede no ser debido a la ya conocida enfermedad neoplásica, sino a un proceso benigno coexistente. Para evaluar la significación de este hallazgo, debe llevarse a cabo una biopsia a cielo abierto. Objetivo: Evaluar las biopsias radioguiadas, y la significación de un foco solitario de hipercaptación costal en pacientes neoplásicos o sospechosos de serlo. Lugar de aplicación: Centro oncológico universitario público. Diseño: Retrospectivo. Población: 23 pacientes con lesiones costales solitarias sospechosas de ser neoplásicas primitivas o bien metastásicas de tumores en actividad en algunos casos, o presumiblemente controlados en otros...

Patologías excepcionales en estructuras de cabeza y cuello / Exceptional pathologies in head and neck structures

De 4711 enfermos con lesiones patológicas de cabeza y cuello tratadas, 14 de ellas (0,29 por ciento) han sido, en nuestra experiencia, variedades únicas en sus respectivos órganos. Siete son presentadas en este artículo.

Enfermedades eosinofílicas cutáneas / Skin eosinophilis diseases

Los eosinófilos son importantes en un grupo de enfermedades cutáneas y frecuentemente constituyen un elemento clásico en la histología de estas entidades. Además, aunque los eosinófilos no sean identificados en los tejidos, participan en la patogenia de otras enfermedades, a través del depósito de sus gránulos. En este trabajo realizamos una revisión de las entidades que comprenden el primer grupo: hiperplasia angiolinfoide con eosinofilia, enfermedad de Kimura, foliculitis pustulosa eosinofílica, síndrome de Wells, granuloma facial y úlcera eosinofílica de la mucosa oral

Granuloma eosinofilo localizado: evolucion pospuncion diagnostica / Eosinophilic bone granuloma: remission after diagnostic biopsy

El proposito de este trabajo es presentar un grupo de 8 pacientes (9 lesiones) con granulomas eosinofilos localizados que, luego de la puncion-biopsia diagnostica y sin mediar ningun acto terapeutico medico o quirurgico, evidenciaron una regresion espontanea de las lesiones. La disminucion del dolor se observo a los 15 dias promedio posteriores a la puncion-biopsia. Utilizando la clasificacion de Womer para definir la evolucion radiologica, se objetivo en los 8 pacientes una remision progresiva a partir del tercer mes. A los 56 meses de seguimiento promedio, ninguno de los pacientes presento dolor ni recidiva radiologica de las lesiones. Si bien han sido publicados multiples esquemas terapeuticos locales y sistemicos para el tratamiento del granuloma eosinofilo, no existe en la literatura medica un criterio unificado. En los 8 pacientes analizados se evidencio una regresion espontanea de estas lesiones luego de efectuar una puncion-biopsia, con franca mejoria clinica e involucion radiografica

Mucormicosis. Reporte de tres casos / Mucormycosis. Report of 3 cases

Mucormycosis is an acute and often fatal infection caused by a fungus of the Mucorales order of the Zygomycetes class. There are various clinical types, usually associated with an underlying disorder. The rhinocerebral mucormycosis is usually seen in diabetics, especially in ketoacidosis, but may also appear in healthy people. We report three cases of mucormycosis diagnosed since april 1987 through january 2001 at the Dr. Domingo Luciani Hospital, Caracas, Venezuela. Two of them had diabetes and one was apparently healthy. The most common clinical presentation of mucormycosis was the rhinocerebral infection, seen in the two diabetic patients, both of them had cavernous sinus thrombosis one with involvement of the carotid artery. The other patient with sinus involvement had no predisposing factors. All patients were treated with amphotericin B and two of them had surgical debridement of involved tissue. We emphasize the importance of an early clinical diagnosis and treatment with surgical debridement of infected tissue combined with amphotericin B.