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.

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.

Úlcera crónica en vestíbulo nasal / Chronic ulcer in nasal vestibule

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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.

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.

Erythematous indurated swelling on nose and upper lip. Cutaneous T cell lymphoma.

A 40-year-old man presented for diagnosis with nodules and plaques of the nose and upper lip, progessive over 1(1/2) years.

Cocaine-induced midline destructive lesions mimicking ENT-limited Wegener's granulomatosis.

The prevalence of cocaine use is rising worldwide, with a resultant rise in associated pathology. Regular nasal use can cause cocaine-induced midline destructive lesions (CIMDL), which can be difficult to distinguish from ear, nose, and throat (ENT)-limited Wegener's granulomatosis (WG). Two cocaine users presented with mid-facial pain, epistaxis, and systemic symptoms. Both had nasal septal perforation, necrosis of sinus mucosa, and positive anti-neutrophil cytoplasmic antibodies (ANCA). Histology was inconclusive and treatment with immunosuppressive drugs was commenced. The first patient continued to use cocaine initially, with improvement in her symptoms only on high doses of steroid. Later she stopped cocaine and this plus a switch from cyclophosphamide to mycophenolate mofetil resulted in successful symptom resolution and steroid withdrawal. The second patient denied cocaine use but having only partially responded to high-dose prednisolone and methotrexate, she admitted continued cocaine use and was lost to follow-up. Evaluation of a patient with destructive lesions of the mid-face should include enquiry about intranasal use of cocaine. Localized ENT involvement, inconsistent ANCA pattern, and atypical biopsy findings for WG should be recognized as features of CIMDL. Although cessation of cocaine use is crucial, there may be a role for immunosuppression.

[Brownish plaques on both sides of the nose]. / Bräunliche Plaques an beiden Nasenseiten.

Granuloma fissuratum (Gf) is painful granular tissue caused by constant pressure exerted by glasses. The differential diagnosis includes further granulomatous dermatoses such as cutaneous sarcoidosis, mycobacterioses and autoimmune diseases. It is also important to rule out a basal cell carcinoma. The simplest and most effective treatment of Gf is to correct the glasses frame or, even better, to avoid wearing glasses to correct eyesight. Topical antiseptic/antibiotic treatment is recommended for superinfected lesions. The Gf usually heals within 1-6 months after correction of the glasses. If the lesion does not heal on its own, complete excision is recommended.

Sialometaplasia necrotizante / Necrotizing sialometaplasia

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Nasal NK/T cell lymphoma presenting as a lethal midline granuloma.

Nasal NK/T cell lymphomas are aggressive, locally destructive, midfacial, necrotizing lesions. The nonspecific clinical symptoms constitute a major stumbling block in the early diagnosis and management of these lymphomas. We report here a case of probable nasal NK/T cell lymphoma in an apparently healthy male that progressed rapidly in a short span of time and was managed subsequently with chemotherapy and external beam irradiation with which the lesion regressed.

Nasal NK/T-cell lymphoma causing diagnostic difficulties.

We present history, clinical presentation and anatomo-pathologic findings of a 24-year-old female patient with a nasal NK/T-cell lymphoma. This rare tumor is characterized by its angiocentric and angiodestructive growth, which results in extensive tumor necrosis. At the first encounter this tumor necrosis made it difficult to identify the nature of the tumor cells. However, this necrosis is a key feature: it is the result of the capacity of neoplastic NK/T-cells to invade vessels. The T-cell character of the neoplastic lymphoid has been shown by immunohistochemitry.

Idiopathic midline destructive disease: fact or fiction.

The differential diagnosis of a progressive destructive lesion of the midface and upper airway region includes both neoplastic and non-neoplastic entities; of these, the majority of cases prove to be either Wegener's granulomatosis or lymphoma. Historically, these sorts of necrotizing midfacial lesions were diagnosed clinically, and as a consequence a variety of overlapping categories of disease sprang up. As pathologic examination of biopsy material became both more widespread and (particularly in the last several years) more sophisticated, many lesions previously thought to be of mysterious origins have proven to be examples of lymphoma (in particular, sinonasal natural killer cell or T cell [NK/T] lymphomas). At present, the evaluation of a patient with a progressive destructive process involving the midface region should include imaging studies (to delineate the extent of disease) as well as biopsy (with sampling of lesional tissue for application of sophisticated testing--including immunohistochemical studies, flow cytometry, or molecular studies as necessary--to exclude the possibility of a NK/T cell lymphoma). There remain occasional patients whose necrotizing midfacial lesions continue to be difficult to classify despite the application of extensive testing; such patients are sometimes described as suffering from the nebulous entity of "idiopathic midline destructive disease". While it remains to be seen whether such patients will ultimately be assigned to other diagnostic groups (as, for example, occult toxic injuries--as in the case of cocaine abusers who are not forthcoming with regard to their drug usage), it seems likely that "idiopathic midline destructive disease" is a diagnostic term of questionable validity which should be used only with extreme reticence in modern practice.

Lethal Epstein-Barr virus associated NK/T-cell lymphoma with primary manifestation in the conjunctiva.

BACKGROUND: Lethal midline granulomas (LMG) are very rare angiocentric NK/T-cell lymphomas in association with Epstein-Barr virus. LMG are reported mainly in East Asia occurring in immune compromised patients. HISTORY AND SIGNS: A 41-year old male patient presented with a conjunctival swelling of his upper left eyelid. The lesion had increased over a period of 2 months despite topical corticosteroid treatment. Conjunctival biopsy revealed a highly malignant, CD3 + and BCL2 + extranodal T-cell lymphoma with features of an NK/T-cell origin (CD56 +, TIA + TCR-rearrangement: germline). All lymphoma cells were positive for Epstein-Barr virus RNA. The proliferation rate was highly elevated at 100 %. THERAPY AND OUTCOME: Systemic 1 (st) cycle chemotherapy with cyclophosphamide, doxorubicin, vincristin and prednisone resulted in a complete remission of the swelling within 4 days. However, one week later a massive conjunctival tumour reappeared with only partial regression after combined chemo- and radiotherapy. The patient died within a month because of untreatable pancytopenia due to malignant bone marrow infiltration. CONCLUSIONS: LMG is a rare but highly malignant Epstein-Barr virus associated NK/T-cell lymphoma that can occur in healthy, immune competent Caucasians. This is the first reported case of an LMG in an immune-competent Caucasian patient with primary ocular manifestation. The LMG has a high mortality rate despite systemic treatment and can be lethal within a few months or even weeks.