1/4. Painful rash and swelling of the limbs after recurrent infections in a teenager: polyarteritis nodosa.polyarteritis nodosa is a rare disease in childhood and adolescence that is difficult to diagnose clinically. We report on a 17-y-old girl presenting with a history of recurrent infections of the upper respiratory tract and conjunctivitis followed by a painful rash on the upper and lower extremities resembling erythema nodosum. The diagnosis of polyarteritis nodosa was proven by skin biopsy. Therapy with intravenous immunoglobulins failed, but with systemic steroids she responded promptly. CONCLUSION: polyarteritis nodosa is a differential diagnosis in adolescents presenting with fever and an erythema nodosum-like rash.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Primary selective deficiency of immunoglobulin m.Selective deficiency of IgM as a primary disorder is seldom encountered1--3, and the syndrome remains ill-defined4. It is generally accepted that for a diagnosis to be made, the serum IgM level should be consistently below 2 SD of normal, no other immunodeficiencies are present, and it is not secondary to other disease processes4. This report of an additional case may provide further insight into this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. pulmonary alveolar proteinosis: relationship to silicosis and pulmonary infection.pulmonary alveolar proteinosis is a rare disease with varied radiographic and clinical manifestations. Thirteen patients are presented and the role of pulmonary lavage in management is illustrated. Six patients had associated diseases such as nocardiosis, leukemia, and silicosis. The broad spectrum of relationships found in alveolar proteinosis suggests that it represents one mechanism by which the lung responds to a variety of insults.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. purpura fulminans.purpura fulminans is a rare disease characterized by cutaneous ecchymosis, hypotension and fever associated with disseminated intravascular coagulation. It usually follows a preparatory infectious illness. A case in a 55 year old woman challenges the concept that this disease is limited to childhood. A review of 100 case reports is presented in which the often quoted high mortality of 90 per cent is found to be no longer tenable.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |