A new case of Erdheim-Chester lipoid granulomatosis is described in a 40-year-old man. In this lipid storage disease, characteristic symmetrical osteosclerotic areas are observed mainly in metadiaphyses of long tubular bones. Bone biopsy is mandatory for definite diagnosis, although characteristic radiographic pictures often provide an important clue to the correct diagnosis.

INTRODUCTION ANCA vasculitis may involve the skin and develop slowly without specific histology, and without autoantibodies. CASE REPORT We report a 50-year-old woman who experienced bilateral mastectomy because of ulcero-necrotic, non-specific inflammatory cutaneous lesions of the breasts. First considered by others as a malinger patient, she developed oto-neurological lesions leading to the...

Affi liations: From the Asthma and Allergy Research Group, Medical Research Institute, Ninewells Hospital and Medical School, University of Dundee. Financial/nonfi nancial disclosures: The author has reported to CHEST the following confl icts of interest: Dr Lipworth has received unrestricted grants from Chiesi Ltd and Teva Pharmaceuticals Industries, Ltd; has acted as consultant to Cipla Limit...

Wegener's granulomatosis is a complex multisystem disease that can be associated with morbidity and mortality. The introduction of cyclophosphamide and glucocorticoids brought about the potential for long-term survival and provided the opportunity and impetus to explore treatment options that can reduce the toxicity of therapy and lessen the likelihood of relapse. With the growth of knowledge r...

A patient with the limited form of Wegener's granulomatosis is reported. The case is unusual because of hilar and mediastinal lymphadenopathy, severe ulceration of the respiratory and digestive tracts, and the rapidly fatal outcome.

We describe an unusual case of Wegener's granulomatosis (WG), in a middle aged, non-smoking female who presented with a lung mass with constitutional symptoms. FNABC from mass revealed a single ill-defined granuloma without necrosis. There was a definite clinical and radiological response to anti-tuberculosis treatment. She was later found to have another mass lesion in nasopharynx. ANCA was ne...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-α, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages...

Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2-8% of patients with granulomatosis with polyangiitis (GPA). Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to lim...

Two patients are presented with Wegener's granulomatosis (WG) and lower respiratory tract infections with Staphyloccus aureus (SA). It is posulated that there is a relationship between the infection and the induction or relapse of the disease. We suggest that bronchoalveolar lavages should be performed in cases of suspected WG to identify SA-infections. The co-existence of WG and SA support the...

Author Affiliations: Department of Ophthalmology, Emory Eye Center, Atlanta, Georgia (Drs Payne, Wells, and Grossniklaus); and Department of Ophthalmology, Cole Eye Institute, Cleveland, Ohio (Dr Srivastava). Correspondence: Dr Grossniklaus, Department of Ophthalmology, Emory Eye Center, 1365 B Clifton Rd, Room BT428, Atlanta, GA 30322 ([email protected]). Author Contributions: All of the autho...