Eosinophilic fascitis: a rare fibrosing disorder.

Eosinophilic fasciitis is a rare systemic disease characterized by inflammatory thickening of the skin and underlying fascia, peripheral eosinophilia, elevated inflammatory markers and typical biopsy findings. Internal organ involvement is rare unlike in other fibrosing disorders. Though this is usually a benign disease, it is often difficult to diagnose and the course may also be variable. Glucocorticoids are the mainstay of therapy, but in many cases steroid sparing immunosuppressive drugs are required particularly if the diseases has protracted course. The author reports a typical case of this rare disorder which could well be a first report from Nepal.

Clinical profile of patients with systemic lupus erythematosus.

INTRODUCTION: Clinical features of systemic lupus erythematosus vary between different parts of the World; however, this information on Nepalese systemic lupus erythematosus does not exist. METHODS: Patients with systemic lupus erythematosus fulfilling the American College of Rheumatology criteria treated in Patan Hospital were studied by means of retrospective review of their case records. The results were compared with various regional and international studies. RESULTS: The female to male ratio was 36:1 with a mean age at diagnosis 28.67 years. Cutaneous manifestations were the most common 72 (96%) presentations followed by musculoskeletal involvement 70 (93%). Haematological and renal involvements each were present in 33 (44%) of patients. Pulmonary and neuropsychiatric involvements were present in 15 (20%) and 8 (11%) patients respectively. Antinuclear antibody and anti-double stranded deoxyribonucleic acid antibodies were positive in 70 (93%) and 66 (88%) of patients respectively. A quarter of patients had some form of co-morbid conditions. At the time of last follow up, 70 (93%), 66 (90%), and 6 (9%) of patients were taking antimalarials, steroids and immunosuppressive agents respectively, mostly in combinations. Eighty seven percent of patients had their disease activity under control at the time of last follow up whereas four percent still had an active disease. In another four percent died during the course of therapy and the outcome was unknown in another four percent of patients who lost to follow up. CONCLUSIONS: Clinical characteristics and antinuclear antibody pattern of Nepalese lupus patients is more or less similar to those with patients from other countries in the region and in the World.

Behçet's disease: an account of three cases.

Behçet's disease is a chronic relapsing inflammatory condition affecting multiple organ-systems of the body. Patient's with this disease present to different specialists with wide spectrum of clinical features of varying severity. Worldwide, this disease is clustered around certain parts of Asia, previously known as 'Silk route'. Here we present three cases of Behçet's disease who presented to our clinic with ocular, dermatological, and articular manifestations.