Pulmonary Langerhans cell granulomatosis is defined as an abnormal Langerhans cells infiltration in the lungs. Multifocal involvement is unusual, however. We report 3 cases of pulmonary Langerhans cell granulomatosis combined with either rib, thyroid or hypothalamus involvement.

BACKGROUND Orbital involvement in Wegener's Granulomatosis (WG) is rare and has an overall good prognosis. CASE A 60-year-old hypertensive Indian female presented with vision loss and painful proptosis of left eye. Orbital incisional biopsy suggested necrotising small vessel inflammation. The saddle nose deformity and pedal nodulo- ulcerative lesions further consolidated the diagnosis of Wege...

Two cases of Wegener's granulomatosis occurred in two women aged 53 and 67 years. The clinical course in both was of rapid deterioration resulting from renal failure and leading to death. At necropsy both cases showed severe and disseminated necrotising granulomatosis in the lungs, kidneys, and spleen but with variable degrees of cardiac and systemic vasculitis. One patient, with appreciable ca...

Wegener's granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener's granulomatosis and multi-organ involvement (upper respiratory tract, lung, kidneys, skin and general symptoms), who showed...

1. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996;39(1):87-92. 2. McDonald TJ, Deremee RA. Wegener's granulomatosis. Laryngoscope. 1983;93(2):220-31. 3. Fauci AS, Haynes BF,...

Necrotizing sarcoid granulomatosis (NSG) is a rare systemic disease characterized by confluent sarcoidlike granulomas with necrotic areas and a variable degree of vasculitis. First described by Liebow in 1973, NSG can be classified as a disease showing features of Wegener granulomatosis and sarcoidosis. The etiology and pathogenesis of NSG are so far unknown; it is considered to be unrelated to...

2. Connors W, Griffiths C, Patl J, Belletrutti PJ. Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease. BMC Gastroenterol. 2014;14:127. 3. Ochi N, Yamane H, Yamagishi T, Monobe Y, Takigawa N. Metotrexate-induced lymphoproliferative disease: Epstein–Barr virus-associated lymphomatoid granulomatosis. J Clin Oncol. 2013;31:e348–50. 4. Chung JH, Wu C, Gilman MD, Palmer...

OBJECTIVE To assess and characterise the risk of bladder cancer, and its relation to cyclophosphamide, in patients with Wegener's granulomatosis. METHODS In the population based, nationwide Swedish Inpatient Register a cohort of 1065 patients with Wegener's granulomatosis, 1969-95, was identified. Through linkage with the Swedish Cancer Register, all subjects in this cohort diagnosed with bla...

BACKGROUND Orofacial granulomatosis is a clinical entity presenting with swelling of the facial and/or oral tissues in association with histologic evidence of noncaseating granulomatous inflammation. Labial swelling is the most common finding. Compromise of the gingival and periodontal tissues may occur but has rarely been described in the literature. Our objective was to characterize granuloma...

CONTEXT Chronic inflammatory vasculitis, namely Wegener's granulomatosis, is an autoimmune disease predominantly affecting the respiratory tract and rarely the mucosa of the upper or lower gastrointestinal tract. We report two cases of isolated pancreatic manifestation of Wegener's granulomatosis, mimicking a tumor. CASE REPORTS Two patients, a 57-year-old man and a 68-year-old woman, present...