[Modern Perspectives on Peripheral Neuropathology].

Recent advances in genetic and antibody testing have limited pathological examination of peripheral nerve specimens. However, when examining peripheral neuropathological findings from a modern perspective, there is often an opportunity to comprehend previously unnoticed observations upon re-examining the same specimen. For example, electron microscopy studies have suggested that the components that distinguish between nodal regions and internodes play a pivotal role in the behavior of macrophages that initiate myelin phagocytosis in the demyelinating form of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). Conversely, some patients previously diagnosed with CIDP were found to possess distinctive mechanisms initiated by autoantibodies against paranodal junction proteins such as neurofascin 155 leading to the emergence of the concept of autoimmune nodopathy. In vasculitis, the roles of neutrophils in antineutrophil cytoplasmic antibody-associated vasculitis, eosinophils in eosinophilic granulomatosis with polyangiitis, and complements in nonsystemic vasculitic neuropathy in tissue damage have also been demonstrated when viewed from a modern perspective. Furthermore, mechanisms attributable to predominant small-fiber loss in hereditary transthyretin amyloidosis have been clarified at an ultrastructural level.

[Bronchial asthma and allergic rhinitis-The skin sample reveals a severe systemic disease]. / Asthma bronchiale und allergische Rhinitis ­ die Hautprobe offenbart eine schwerwiegende Systemerkrankung.

This article reports the case of a 30-year-old female patient who suffered for many years from initially unspecific symptoms, such as recurrent, nonallergic and noninfectious sinusitis, late-onset bronchial asthma and pronounced lymphadenopathy; however, the correct diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) could only be made by histological investigations after the appearance of skin symptoms. The EGPA is a severe systemic disease which, if left untreated, can cause multiple organ damage and even be fatal. With adequate treatment the disease is mild in more than 90% of cases and patients can even completely recover. By making the correct diagnosis, the patient could be successfully treated and the risk of late manifestations and subsequent damage with a potentially fatal outcome was reduced.

Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis

Abstract Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions.

Granulomatosis eosinofílica con poliangeítis: reporte de caso yrevisión de la literatura / Eosinophilic granulomatosis with polyangiitis: case report and review of the literature

La granulomatosis eosinofílica con poliangeítis (Síndrome de Churg-Strauss) es una enfermedad vasculítica primaria poco frecuente. El diagnóstico actualmente se define a partir de la presencia de al menos cuatro de seis criterios propuestos por la Sociedad Americana de Reumatología, los cuales incluyen: asma bronquial, eosinofilia mayor que 10 por ciento, sinusitis paranasal, infiltración pulmonar, evidencia histológica de vasculitis y compromiso neurológico ya sea mono o polineuropático. En el presente artículo se reporta el caso de un paciente de 56 años con antecedentes de asma bronquial, rinitis alérgica y poliposis nasal operada, derivado a nuestro centro por cuadro de aumento de volumen doloroso en ambas extremidades inferiores, baja de peso, parestesias y debilidad muscular. Asociado a esto desarrolló lesiones purpúricas palpables cuya biopsia resultó compatible con granulomatosis eosinofílica con poliangeítis. El paciente posteriormente recibió tratamiento inmunosupresor con prednisona y un pulso de ciclofosfamida con buena respuesta clínica. Se presenta una revisión bibliográfica a propósito del caso.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) is an uncommon primary vasculitis. The diagnosis is currently defined by the presence of at least four of six criteria proposed by the American College of Rheumatology, which include: asthma, eosinophilia less than 10 percent, paranasal sinusitis, pulmonary infiltration, histologic evidence of vasculitis and neurologic compromise as mono or polyneuropathy. In the present article, we report the case of a 56 year-old man with history of asthma, allergic rhinitis and operated nasal polyposis, referred to our center with painful bulking in both lower extremities, weight loss, paresthesias and muscle weakness. It also developed palpable purpura. Biopsy of skin lesions was compatible with eosinophilic granulomatosis with polyangiitis. The patient subsequently received immunosuppressive therapy with prednisone and a cyclophosphamide bolus with good clinical response. A review on the subject is also presented.

Vasculitis con lesiones en cuero cabelludo como única manifestación cutánea / RF-Rituximab: Revolutionizing the Treatment of Pemphigus

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Colecistitis aguda alitiásica como manifestación inicial de una granulomatosis eosinofílica con poliangeítis (Churg-Strauss). A propósito de un caso / Acute acalculous cholecystitis as the initial manifestation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). A case report

La granulomatosis eosinofílica con poliangeítis (Churg-Strauss) es una vasculitis sistémica con inflamación granulomatosa necrosante, rica en eosinófilos, a menudo con afectación del tracto respiratorio y vasculitis necrosante que afecta vasos pequeños y medianos, asociado con asma y eosinofilia. Presentamos un paciente cuya manifestación inicial de la enfermedad fue una colecistitis aguda alitiásica por vasculitis. Aunque la colecistitis aguda alitiásica como síntoma inicial de la granulomatosis eosinofílica con poliangeítis es muy infrecuente, se debería tener en cuenta en el diagnóstico en pacientes con dolor abdominal de origen desconocido con eosinofilia, asma o rinitis alérgica (AU)

Eosinophilic granulomatosis with polyangiitis (Churg-Stauss) is a systemic vasculitis with eosinophil-rich and necrotizing granulomatous inflammation and necrotizing vasculitis predominantly affecting small to medium vessels. It often involves the respiratory tract and is associated with asthma and eosinophilia. We describe a case in which acute acalculous cholecystitis was the initial manifestation of the disease. Although acute acalculous cholecystitis rarely appears as an early manifestation in eosinophilic granulomatosis with polyangiitis, this entity should be taken into consideration in the differential diagnosis of patients with abdominal pain of unknown origin and a history of eosinophilia, asthma, or allergic rhinitis (AU)

Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 percent, platelets = 260,000, white blood cells = 8300, 74 percent segmented, 4 percent eosinophils, 19 percent lymphocytes and 3 percent monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result

Síndrome de Churg-Strauss de aparición precoz / Early-onset of Churg-Strauss syndrome

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Dermatitis granulomatosa intersticial con artritis / Interstitial granulomatous dermatitis with arthritis

La dermatitis granulomatosa intersticial con artritis es una enfermedad sistémica que afecta a la piel y al sistema musculosquelético. Esta nueva entidad se caracteriza por formar cordones en disposición lineal o arciforme, aunque se ha descrito un amplio espectro de presentaciones clínicas. Histológicamente se aprecia un infiltrado intersticial difuso con histiocitos y focos de degeneración del colágeno, así como la formación de pequeños granulomas alrededor de estas áreas de degeneración. Además existe un número variable de leucocitos polimorfonucleares en el infiltrado. Nosotros describimos dos nuevos casos con sus características clínicas e histopatológicas que se enmarcan dentro del espectro de presentación de esta patología. También realizamos el diagnóstico diferencial con el granuloma anular intersticial y comentamos los últimos artículos publicados en la literatura médica (AU)

Sindrome de Cronkhite-Canada: presentacion de un caso / Cronkhite-Canada syndrome: report of a case

Se presenta una paciente del sexo femenino de 71 años con antecedentes de una gastrectomía parcial por gastritis erosiva y que desarrolla un síndrome de Cronkhite-Canada con sudopoliposis gastrointestinal generalizada, hiperpigmentación de la piel, onicodistrofia y alopecía. Presentaba diarrea severa con intensa hipooalbuminemia y alteraciones asociadas inusuales con hiperelasticidad cutánea e hiperextensibilidad articular en los dedos. Se observó remisión parcial del síndrome luego de intenso tratamiento dietético y de desinfección con antibióticos del tracto gastrointestinal

Angeitis alérgica granulomatosa

Presentamos el caso de una mujer de 38 años con asma, eosinofilia periférica, sinusitis y opacidades interticiales difusas, bilaterales y fugaces en la radiografía de tórax. El procedimiento diagnóstico fue biopsia pulmonar a cielo abierto donde se encontró una vasculitis eosinofilica características del Síndrome de Churg-Strauss. Comentamos los principales hallazgos clínicos, radiológicos e histopatológicos.

Angeítis y granulomatosis alérgica (Síndrome de Churg-strauss). Presentación de un caso sin asma

Se presenta un caso comprobado de síndrome de Churg-Strauss, el tercero que se aplica en el país, con dos características clínicas interesantes: ausencia de asma bronquial y atopia y compromiso predominate a nivel del aparato gastrintestinal. Se hace notar que la ausencia de asma no excluye el diagnóstico.