Characteristics of histamine-releasing activity in the sera of patients with chronic idiopathic urticaria.

BACKGROUND: The serum histamine-releasing activity (HRA) found in a sizable percentage of patients with chronic idiopathic urticaria (CIU) has been partially characterized. However, the variable effect of individual HRA+ sera in basophils of different donors and the relationship of HRA to the clinical course require further investigation. OBJECTIVE: The study was performed to characterize the HRA found in sera of some members of a sizable group of carefully evaluated patients with CIU. METHODS: Sera of 70 patients with CIU, evaluated with a standard protocol, were screened for increased HRA. HRA+ sera were fractionated, heated, and tested on unaltered and altered basophils obtained from a panel of normal donors. HRA levels were compared with concomitant clinical manifestations. RESULTS: HRA+ sera were found in 30% of our patients with CIU, HRA was predominantly in the IgG fraction, sensitive to 56 degrees C heating for 4 hours, and generally reacted more with IgE-stripped basophils. Considerable variation in the degree of response to HRA+ sera in the basophils of different normal subjects did not correlate with the degree of response of these cells to heterologous anti-IgE antiserum. Serum HRA levels were generally much lower when symptoms decreased in these patients with CIU. CONCLUSION: Serum HRA from patients with CIU appears to bind most commonly to the IgE receptor and may be a marker of clinical disease activity. HRA appears in an IgG-containing fraction of the serum and may contain IgE in some cases.

Comparison of serum histamine-releasing activity and clinical manifestations in chronic idiopathic urticaria.

We found increased serum histamine-releasing activity (HRA) in 27% of patients with chronic idiopathic urticaria. Patterns of clinical manifestations were similar in those with and without serum HRA, including responses to a standard treatment regimen. In HRA+ patients the degree of serum HRA generally correlated with clinical disease activity.

Serum tryptase in idiopathic anaphylaxis: a case report and review of the literature.

Anaphylaxis is a life-threatening disease that characteristically presents with multiple arrays of dermatologic, respiratory, cardiovascular, and gastrointestinal derangements, in general, suddenly after exposure to an allergen. It can, however, occur without an identifiable precipitant or event, and this well-defined entity has been called idiopathic anaphylaxis. The diagnosis of idiopathic anaphylaxis is made after an appropriate allergic evaluation and exclusion of a provocative trigger. We report an unusual case of manifesting with gastroenteritis, urticaria, hypotension, and syncope. Measurement of serum tryptase, a mast cell enzyme, was used to substantiate the diagnosis. Tryptase level is a useful test that can be used to help diagnose this potentially fatal disease.

Polyautoimmune syndrome in common variable immunodeficiency.

Common variable immunodeficiency (CVI) is a heterogenous disorder with hypogammaglobulinaemia and multiple bacterial infections primarily involving the sinopulmonary tract. CVI patients have been known to have an increased tendency to develop autoimmune manifestations. Commonly associated autoimmune diseases include immune thrombocytopenic purpura, autoimmune haemolytic anaemia, and rheumatoid arthritis. In this paper we report a case of CVI presenting with multiple unusual autoimmune diseases including parotitis, vitiligo, atrophic gastritis, pernicious anaemia, and primary biliary cirrhosis. To our knowledge, this is the first case of CVI with polyautoimmunity and antimitochondria antibody. Recognition of this association is important because early diagnosis and treatment can greatly influence the prognosis.

POEMS syndrome presenting with cardiomegaly and cardiomyopathy.

A rare association of multisystemic manifestations with plasma cell dyscrasia has been termed POEMS syndrome, which includes polyneuropathy, organomegaly, endocrinopathy. M-protein, and skin changes. From literature reports, organomegaly consists of hepatosplenomegaly and/or lymphadenopathy. We here present a case of POEMS syndrome that had most of the typical features of POEMS, except that organomegaly only involved the heart. The patient's multiple clinical manifestations, including cardiomyopathy, improved with chemotherapy, which is characteristic of POEMS syndrome. However, cardiomegaly or cardiomyopathy have not previously been described with POEMS. The present case suggests that cardiomegaly and cardiomyopathy may be added to the organomegaly in POEMS.

Cutis verticis gyrata.

When the scalp exhibits folds and furrows resembling gyri, it is termed cutis verticis gyrata. The possible etiologies may be categorized as primary essential, primary nonessential, and secondary cutis verticis gyrata. This is based on history, physical examination, and histologic criteria with or without laboratory examinations.