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.

Churg-strauss syndrome: a rare systemic vasculitis

Churg-Strauss syndrome is a rare diffuse small/medium sized vessel vasculitis that is almost invariably accompanied by severe asthma. The cause is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. We report here a middle aged lady who presented with sudden onset of vasculitic skin lesions in association with long standing asthma. Skin biopsy and serology revealed diagnosis of Churg-Strauss syndrome. She was successfully managed with oral prednisolone

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

Churg-Strauss syndrome with perforating ulcers of the colon

We report a case of a 72-year-old woman with Churg-Strauss syndrome, who presented with intestinal perforation. She has had bronchial asthma with peripheral blood eosinophilia for 30 years. Gross findings of a resected colon showed multiple ulcers with perforation. Histologic findings demonstrated transmural inflammation infiltrated with large numbers of eosionophils, neutrophils and lymphoplasma cells, and characteristic extravascular granuloma in the subserosa. There were multifocally-distributed transmural vasculitis showing all stages of activity in medium and small-sized arteries and veins located in the submucosa, and proper muscle and subserosal layers of the colon, some of which revealed granulomatous inflammation. Histologic finding of liver showed chronic viral hepatitis B with mild inflammatory activity and macronodular cirrhosis. Immunohistochemical findings, acid fuschin orange G staining and electromicroscope found no evidence of hepatitis B virus infection contributing to the pathogenesis of this lesion.

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.

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