Cocaïne et lésions destructrices centro-faciales : à propos d'un cas.

INTRODUCTION: Cocaine use is associated with multiple complications, some of which can mimic systemic diseases, especially Antineutrophil Cytoplasmic Antibody (ANCA) associated vasculitis. We report a case of Cocaine Induced Midline Destructive Lesions (CIMDL) for which a diagnosis of granulomatosis with polyangiitis (GPA) was discussed. CASE REPORT: A 42-year-old male, cocaine consumer, was admitted in our department for a centrofacial destructive process. He had no extra ear, nose and throat (ENT) involvement. ANCA were positive with a perinuclear fluorescence pattern and an anti-Proteinase 3 specificity. Regarding this unusual immunologic pattern and in the absence of histological argument for a GPA, a diagnosis of CIMDL was made. CONCLUSION: CIMDL is a centrofacial destructive process due to intranasal cocaine use. It is frequently associated with the presence of p-ANCA with both anti-HNE and anti-PR3 specificity.

Accurate diagnose and management of advanced nasal type extranodal nk/t cell lymphoma. a case report

ABSTRACT Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare entity in otorhinolaryngology. Its management requires skilled physicians in order to suspect this disease and making a proper diagnosis at early stages. This paper reports the case of a 31-year-old male patient, with one month of nasal obstruction, recurrent sinusitis, palatal ulceration and a necrotizing lesion. Histopathology reported lymphoid infiltrate polymorph angiocentric growth pattern and extensive areas of necrosis. Immunohistochemistry confirmed the phenotype for T/NK cells: positive CD3, BCL2, CD4 and CD56. IgG for Epstein-Barr virus was also positive. The initial staging was T4, N1, M0, Eastern Cooperative Oncology Group (ECOG) scale was 1, with intermediate risk, and low International Prognostic Index (IPI); based on this results, the patient was referred to oncology to initiate treatment. After a ten-month follow-up, the patient's condition improved, with complete remission of nasal and palate injuries; no relapse has occurred to date. This case is a clear example of the importance of early diagnostic through multiple biopsies in order to establish a specific treatment to decrease complication rates and improve prognosis.

Caracterización de lesiones letales de la línea media facial en pacientes del Hospital Escuela Universitario, Honduras entre 2011-2014 / Characterization of lethal injuries of the facial midline in patients of the University School Hospital, Honduras between 2011-2014

Objetivo: Caracterizar lesiones letales de línea media facial, signos y síntomas frecuentes, género y procedencia de los pacientes, histopatología e inmunohistoquímica en base a registros médicos institucionales de HEU entre 2011 y 2014.La lesión letal de línea media es un síndrome que inicialmente engloba variadas entidades: Linfomas no Hogdkin de células NK y T, Linfomas no Hogdkin de células B, enfermedades autoinmunes como la Granulomatosis con Poliangeítis, muchas causas infecciosas e idiopáticas con destrucción acelerada y catastrófica de la región nasofaríngea, senos paranasales y septum nasal. Síndromes de difícil diagnóstico con enfoques terapéuticos muy distintos. Metodología. Se realizó un estudio descriptivo, transversal, con revisión de todos los registros de biopsias realizados en el departamento de Anatomía Patológica del HEU desde el año 2011 al 2014. Cumplen criterios de inclusión, 34 casos. Resultados: Mayor prevalencia de lesiones en hombres 59 %, dentro del rango de edad de 19 a 59 años, con predomino de la región central de Honduras. Signo más frecuente: masa obstructiva. Diagnóstico más consignado fue Linfoma No Hodgkin sin especificación. Conclusión: Frecuencia de lesiones letales de la línea media es mayor en varones, procedentes en su mayoría de región central, síntoma y signo más frecuentes son masa obstructiva con ulceración y la rinorrea purulenta; la utilización de marcadores de inmunohistoquímica es deficiente para definir los casos inespecíficos de Linfoma No Hodgkin Nasales.

Objective: To characterize lethal facial midline lesions, frequent signs and symptoms, gender and origin of the patients, histopathology and immunohistochemistry based on HEU institutional medical records between 2011 and 2014. Lethal midline injury is a syndrome that initially encompasses a variety of entities: non-Hogdkin lymphomas of NK and T cells, non-Hogdkin B-cell lymphomas, autoimmune diseases such as granulomatosis with polyangiitis, many infectious and idiopathic causes with accelerated and catastrophic destruction of the nasopharyngeal region, paranasal sinuses and nasal septum. Syndromes which are difficult to diagnose with very different therapeutic approaches.Methodology. A descriptive, crosssectional study was carried out with a review of all biopsy registries performed in the Department of Pathological Anatomy of HEU from 2011 to 2014. 34 cases meet the inclusion criteria. Results: There was a higher prevalence in men 59%, within the age range of 19 to 59 years, with predominance of the central region of Honduras. Most frequent sign: obstructive mass. Most diagnosed was Non-Hodgkin's lymphoma without specification. Conclusion: Frequency of lethal midline lesions is greater in males, mostly from the central region. The most frequent symptoms and signs are obstructive mass with ulceration and purulent rhinorrhea; the use of immunohistochemical markers is deficient to define nonspecific cases of Nasal Non-Hodgkin's Lymphoma.

Granuloma letal de la línea media como diagnóstico diferencial de la vasculitis de Wegener: presentación de una paciente

Entre las lesiones que afectan la línea media se encuentra el granuloma letal olinfoma nasal de células T/natural killer; es una enfermedad agresiva y poco frecuente. Se presenta una paciente a la que, tras manifestar síntomas de vías respiratorias superiores, se le diagnóstico una vasculitis de Wegener, pero la evolución posterior y la revaloración clinicopatológica confirmaron otraenfermedad: el granuloma letal de la línea media(AU)

Úlcera crónica en vestíbulo nasal / Chronic ulcer in nasal vestibule

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Lesión necrotizante de la línea media con fístula oronasal causada por inhalación de cocaína / Cocaine-induced midline destructive lesion and oronasal fistula

Los autores realizan una revisión bibliográfica de las perforaciones palatinas asociadas a lesiones necrotizantes de la línea media por inhalación de cocaína y, aportan un caso clínico propio con fístula oronasal. La frecuencia de las perforaciones palatinas asociadas a las lesiones necrotizantes de la línea media por inhalación de cocaína es muy superior a las encontradas en la granulomatosis de Wegener. La presencia de una perforación palatina es también un dato sugestivo de linfoma extranodal. La clave diagnóstica de todas las lesiones necrotizantes de la línea media es histopatológica pero obtener un diagnóstico inequívoco es más difícil de obtener de lo que podría suponerse (AU)

The authors perform a literature review of palatal fistulas present in midline necrotizing nasal lesions due to inhalation of cocaine and present a case report. The frequency of palatal fistulas associated with midline necrotizing lesions by inhalation of cocaine is superior to tose found in Wegener's granulomatosis. The presence of a palatal fistula is also suggestive of a extranodal lymphoma. Histopathology is the key to the differential diagnosis but getting an unequivocal diagnosis is more difficult to obtain than might be supposed (AU)

Extranodal natural killer/T-cell lymphoma, nasal type: 'midline lethal granuloma.' A case report.

Extranodal natural killer/T cell lymphoma, nasal type, is a non-Hodgkin lymphoma, most commonly affecting the nasal cavity, paranasal sinuses and nasopharynx. Clinically it is characterised by destruction of facial tissues, commencing in the midline. In most cases it arises from malignant transformation of natural killer cells (NK); sometimes from malignant transformation of cytotoxic T cells.Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.

Síndrome do granuloma letal da linha média: um dilema diagnóstico / Lethal midline granuloma syndrome: a diagnostic dilemma

A rara síndrome do granuloma letal da linha média apresenta difícil diagnóstico, em razão da grande variedade de doenças que podem causá-la e um desconhecimento pela maioria da classe médica. No presente artigo relatamos caso de paciente com esta doença, provocada por carcinoma epidermoide, chamando a atenção para os diagnósticos diferenciais e aspectos clínico-radiológicos que podem auxiliar no diagnóstico.

The rare lethal midline granuloma syndrome is difficult to diagnose because of the wide array of related diseases and lack of knowledge by the majority of physicians. In the present report, the authors describe the case of a patient with this disease, caused by squamous cell carcinoma, drawing attention to differential diagnoses and to clinical and radiological findings that may be useful to define the diagnosis.

Mucor irregularis infection and lethal midline granuloma: a case report and review of published literature.

Mucor irregularis (Rhizomucor variabilis) infection and lethal midline granuloma (LMG) are characterized by progressive swelling, ulceration, and destruction of the central face that is usually fatal. Pathological features are inflammation, necrosis, and granulation. LMG has been called by various names, and in recent years, it has been known as NK/T cell lymphoma. However, diagnosis still relies on the progressive necrosis course rather than malignancy in histology. The disease has long challenged physicians, particularly when it worsens with radiotherapy or chemotherapy but sometimes achieves total remission without anti-malignancy therapies. We describe a 35-year-old man who had typical clinical-pathological symptoms of LMG, which turned out to be primary M. irregularis infection; that was diagnosed by positive tissue culture and fungal elements in histology. The patient was successfully treated with antifungal therapy (liposomal amphotericin B, total 4,600 mg and amphotericin B total 277 mg, over a duration of 70 days). We hereby review current knowledge about the epidemiology, clinical manifestations, radiographic characteristics, and pathologic features of LMG with those of M. irregularis infection and their associations. We conclude that primary M. irregulars infection can mimic the clinico-pathological symptoms of LMG and the condition responds favorably to aggressive antifungal therapy.

Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener / Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC), que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW) nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC) dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.

Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis.

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.

The dilemma of midline destructive lesions: a case series and diagnostic review.

BACKGROUND: Midline destructive lesions (MDLs) of the nose are a diagnostic dilemma due to an extensive differential diagnosis and vague presenting signs and symptoms. Etiologies may be neoplastic, autoimmune, traumatic, infectious, or unknown. STUDY DESIGN: Case series and review of the literature were done. METHODS: Medical records of 8 patients presenting with an MDL were reviewed. RESULTS: Each patient received nasal endoscopy, computed tomography scan of the sinuses, laboratory workup, culture (aerobes, anaerobes, fungus, and acid-fast bacilli), and biopsy with flow cytometry. Laboratory tests included complete blood count, basic metabolic panel, erythrocyte sedimentation rate, angiotensin-converting enzyme, antineutrophil antibodies, rheumatoid factor, anti-Ro and anti-La antibodies, Epstein-Barr virus antibodies, coccidiomycosis serology, HIV antibodies, fluorescent treponemal antibody absorption, classic antineutrophil cytoplasmic antibodies, perinuclear antineutrophil cytoplasmic antibody, proteinase 3, and myeloperoxidase. Choice of diagnostic study was individualized for each patient. Two patients were diagnosed with natural killer/T-cell lymphoma, 2 were diagnosed with Wegener's granulomatosis, and 4 remained idiopathic, despite the extensive workup. A diagnostic algorithm to aid in the approach to MDLs is presented. CONCLUSIONS: The diagnosis of MDLs remains difficult but is aided by a systematic approach and familiarity with multiple diagnostic techniques. It is imperative to take multiple tissue specimens from various sites, send them fresh, and communicate suspicion of lymphoma. Despite diagnostic advances and improved understanding of the diseases underlying MDLs, an etiology is often not identified.

Extranodal NK/T cell Lymphoma, nasal type: clinical, radiological, histological features for early diagnosis

Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.