1/15. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy.A 55-year-old woman with a past history of lower respiratory tract infections presented with a photosensitive eruption. Polymorphic light eruption (PLE) was diagnosed on the basis of the temporal relationship to sun exposure and the diagnosis was supported by positive monochromator irradiation tests in the ultraviolet A wavelength spectrum. Investigation of the patient's immune status identified low levels of all immunoglobulin (Ig) subtypes consistent with common variable hypogammaglobulinaemia. Intravenous Ig replacement therapy, instituted to minimize risks from bacterial infections, was commenced and over the ensuing months resulted in a complete resolution of the PLE. PLE is considered to represent a type IV hypersensitivity reaction directed against a cutaneous autoantigen induced by exposure to ultraviolet light. In PLE, nonspecific immunomodulatory mechanisms of intravenous Ig may be active, such as a reduction in the synthesis of cytokines and a blockage of the IgG Fc receptors on macrophages.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
2/15. Clonal CD8 infiltration of the skin in common variable immunodeficiency: a prelymphomatous stage?common variable immunodeficiency is a heterogeneous syndrome characterized by hypogammaglobulinemia and recurrent bacterial infections. patients with this syndrome also have an increased incidence of autoimmune disease and malignancy, most notably lymphoproliferative disorders. The treatment for common variable immunodeficiency is supported by the use of intravenous infusion of immunoglobulins that allows for control of the disease and avoidance of recurrent opportunistic infections. This report describes a young patient having common variable immunodeficiency who presented with a clonal CD8( ) lymphocytic infiltration of the skin clinically manifesting as a widespread papulonodular eruption and resolving with intravenous immunoglobulin therapy. We speculate that such a unique cutaneous presentation may represent a prelymphomatous condition because of the development of an autonomous T-suppressor/cytotoxic cell clone, possibly also responsible for the impaired immunoglobulin production.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
3/15. Cutaneous granulomas masquerading as tuberculoid leprosy in a patient with congenital combined immunodeficiency.Combined immunodeficiency disorders are characterized by abnormalities in cellular and humoral immunity. This classification includes common variable immunodeficiency (CVI), a primary immunodeficiency disorder characterized by hypogammaglobulinemia, recurrent bacterial infections, and significant T-cell abnormalities. Associated autoimmune diseases include rheumatoid arthritis, pernicious anemia, idiopathic thrombocytopenic purpura, and systemic lupus erythematous. Granulomatous lesions in lymphoid tissues, solid organs, and skin have been reported. We describe a patient with CVI who developed cutaneous granulomas with perineural invasion; to our knowledge, this is a previously undescribed feature.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
4/15. mycobacterium avium intracellulare otitis media.Atypical mycobacterial infections of the middle ear are extremely rare. To our knowledge, only eight cases have been reported in the literature, five of which involve mycobacterium avium intracellulare. We present a case of culture-proven, M. avium intracellulare otomastoiditis in an 8-year-old boy with common variable immunodeficiency syndrome. The patient clinically presented with pain and otorrhea. The histopathology was marked by acid-fast bacilli-laden histiocytes. Consideration of this entity in the differential diagnosis of chronic, recalcitrant otorrhea can lead to timely diagnosis, treatment, an decreased morbidity.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
5/15. Myofibroblastic tumors involving bilateral adrenal glands and skin in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID) is a heterogeneous form of primary immune deficiency characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. In addition to recurrent infections, patients with this syndrome have an increased incidence of autoimmune diseases and malignancy. A patient with CVID in whom myofibroblastic tumors affecting both adrenal glands and skin is presented.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/15. Gammadelta T lymphocytosis associated with granulomatous disease in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID) is a heterogeneous group of immunodeficiency syndromes that involves defective production of specific antibodies and decreased serum concentrations of > or =1 immunoglobulin isotype. We describe a patient with an atypical case of CVID who had extensive granulomatous lesions that were partially attributable to mycobacterial infection. In the peripheral blood, there was a massive increase in the number of double-negative CD3 T cells that expressed the gammadelta T cell receptor.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
7/15. common variable immunodeficiency and breast cancer.We describe two women with early onset breast cancer (at ages 31 and 38) who were found to have common variable immunodeficiency (CVID). Both women had a history of frequent and unusual bacterial infections. The incidence of breast cancer in young women and of CVID are both low, and the coincident diagnosis in two patients from a relatively small population base suggests the possibility of a pathophysiologic or causal relationship.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
8/15. Cardiac conduction abnormalities and congenital immunodeficiency in a child with Kabuki syndrome: case report.BACKGROUND: Since it's recognition in 1981, a more complete phenotype of Kabuki syndrome is becoming evident as additional cases are identified. Congenital heart defects and a number of visceral abnormalities have been added to the typical dysmorphic features originally described. CASE REPORT: In this report we describe the clinical course of a child diagnosed with Kabuki syndrome based on characteristic clinical, radiological and morphologic features who died of a cardiac arrhythmia at 11-months of age. This infant, however, had abnormal pulmonary architecture and alterations in his cardiac conduction system resulting in episodes of bradycardia and asystole. This child also had an immunological phenotype consistent with common variable immunodeficiency. His clinical course consisted of numerous hospitalizations for recurrent bacterial infections and congenital hypogammaglobulinemia characterized by low serum IgG and IgA but normal IgM levels, and decreased antibody levels to immunizations. T-, B- and NK lymphocyte subpopulations and T-cell function studies were normal. CONCLUSION: This child may represent a more severe phenotype of Kabuki syndrome. Recurrent infections in a child should prompt a thorough immunological evaluation. Additionally, electrophysiology testing may be indicated if cardiopulmonary events occur which are not explained by anatomic defects.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
9/15. common variable immunodeficiency mimicking rheumatoid arthritis with sjogren's syndrome.Several autoimmune diseases have been reported to be associated with common variable immunodeficiency disease (CVID), including rheumatoid arthritis and sjogren's syndrome. On the other hand, approximately 20-30% of patients with rheumatoid arthritis develop secondary sjogren's syndrome. A 26-year-old woman had a 6-year history of chronic symmetric polyarthritis and 3-year history of sicca syndrome prior to admission for pneumonia. Rheumatoid arthritis with secondary sjogren's syndrome had been diagnosed 1 year before. The patient had experienced 3 episodes of pneumonia during the previous 3 years. Markedly depressed serum immunoglobulin levels prompted a suspicion of common variable immunodeficiency, and the impression was confirmed after a series of examinations. Monthly administration of intravenous immunoglobulin (IVIG) alleviated the polyarthritis and improved the sicca syndrome. IVIG replacement therapy was ultimately successful in curing recurrent bacterial infections, chronic polyarthritis, and improving the severity of sicca syndrome.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
10/15. Etanercept treatment of cutaneous granulomas in common variable immunodeficiency.common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, poor antibody responses, and recurrent bacterial infections, usually of the sinorespiratory tract. A not uncommon complication is granuloma of the lungs, spleen, liver, and/or skin. We report the case of an 18-year-old boy with CVID and chronic granulomas of the left arm (since 13 years of age) refractory to treatment with antibiotics, intravenous immunoglobulin, antifungal agents, systemic and intralesional steroids, IFN-gamma, cyclosporine, methotrexate, hydroxychloroquine, localized radiation therapy, and surgical excision. The lesions improved after treatment with the systemic administration of the TNF-alpha inhibitor etanercept for 1 year. Etanercept prevents soluble TNF from binding to its cell membrane receptor, leading to inhibition of its inflammatory cascade. We recommend further trials of etanercept in patients with CVID with noninfectious recalcitrant granulomas.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
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