[Hyper-IgM syndrome: mucocutaneous lesions and neutropenia]. / Síndrome de hiper-IgM: lesiones mucocutáneas y neutropenia.

The hyper-IgM syndrome is a rare immunodeficiency disease in which the ability of B cells to switch immunoglobulin production from IgM to IgG, IgA and IgE is defective. The deficiency of IgG and IgA leads to recurrent infections of the respiratory tract, but some patients are susceptible to infection with opportunistic microorganisms, such as Cryptococcus neoformans, and also are prone to neutropenia and mucocutaneous ulcerations. We report a case of a two-year-old boy that was given the diagnosis of the hyper-IgM syndrome on the basis of low serum concentrations of IgG and IgA and high serum levels of IgM associated with C. neoformans infection, neutropenia and mucocutaneous ulcerations. Intravenous immune globulin improves dramatically the disorder, including neutropenia. To date, periodical infusion of immune globulin has prevented the development of serious infections.

[Treatment of lupus crisis with IgIV]. / Tratamiento de crisis lúpica con IgIV.

BACKGROUND: Intravenous Immunoglobulin (i.v.Ig) has been advocated as efficacious for Systemic Lupus Erytematosus (SLE) and Lupic Nephritis (LN) using high dosages, propitious a prolonged remission of SLE. MATERIAL AND METHOD: We show the case of a male patient 16 years old, with SLE diagnosis until 1993, and LN phase IV. He attend to hospital with a Lupic Crisis, positives ANAs, DNAds, ScL 427, C3 45, C4 13, IgG 179, urinary sediment: leukocytes 30-40 xc, erythrocytes 8-10 xc. Renal failure: BUN 243, seric Cr: 10.16. I.v. Ig administrated 400 mgs k do. And nephrologic assistance, NK. RESULTS: There was improvement after infusion, clinical and serological, persisting with renal failure. The mechanism by which i.v. Ig might have effected improvement in this patient was reviewed. CONCLUSION: The use in our patient with SLE and LN was satisfactory. Is difficult affirm categorical the outcome or failure of i.v. Ig in patients with SLE, even report founded are only few cases report.