Selective immunoglobulin M deficiency associated with disseminated molluscum contagiosum

Abstract

A 16-year-old girl with disseminated molluscum contagiosum (MC) was found to have a very low level of serum IgM, elevated levels of IgG and IgA, and a high level of IgE. She had normal numbers of peripheral blood IgM+, IgG+ and IgA+ B-lymphocytes but their terminal differentiation into plasma cells could not be induced by pokeweed mitogen (PWM) in vitro. On the other hand, the patient's T-cells showed normal helper functions in the PWM system and normal interferon (IFN) production in vitro. However, the IgM+ B-cells can be induced to differentiate into IgM secreting cells by Epstein-Barr virus (EBV), suggesting that the genetic mechanism for synthesis of the component immunoglobulin proteins is present. T-cell functions were impaired, as shown by delayed type cutaneous hypersensitivity (DTH) and mitogen response. The data suggest that the selective IgM deficiency of the patient is due mainly to defects in B-cells at the terminal differentiation stage, but immunological abnormalities are present in both B and T-cell systems. Neutrophil functions examined were normal. MC was treated by intravenous injection of IFN without any side effects; however, no clinical improvement was achieved.

Pediatric Selective Igm Immunodeficiency

“…Increased IgM-specific T cell suppressor function [35,40,41]; normal T cell suppression function [42][43][44] Excessive isotype nonspecific T cell suppressive activity [40]; increased IgM specific suppressive T cell function [35] Defect in T cell help [10,26]; normal T cell help [17,42,44,45] Nonspecific T cell abnormalities [26]; normal T cell function [42] B cell function Defect in B cell differentiation into IgM-immunoglobulin secreting cells [40,46,47] T and B cell enumeration and phenotype…”

“…Normal peripheral T and B cell phenotypes [42,43,47,48]; increased CD8+ cells and inverted CD4/CD8 ratios [40]; increased CD4+ cells and decreased CD8+ cells [47] Reduced number of IgM secreting B cells [45,46] with a failure of secreted mu mRNA synthesis [46]; normal surface IgM expression on B cells [10,17,35,43,[47][48][49]; normal secreted mu mRNA synthesis [49] Mitogen/antigen stimulation Mitogen and antigen stimulated B cell proliferation assays with normal IgM responses [11,42]; decreased antigen proliferation IgM responses [4,17,40,41,[45][46][47][48][49] Deficient IgM responses to viral antigens and/or endotoxin containing vaccines and deficient isohemagglutinin antibodies [24] Failure to respond to antigen challenge with tetanus toxoid, pneumococcal vaccine, meningococcus vaccine, Salmonella O and H antigens, and typhusparatyphus vaccine [10-12, 19, 42, 50] Complement No complement deficits [10] Delayed hypersensitivity Reduced delayed cutaneous hypersensitivity [10,17]; normal delayed cutaneous hypersensitivity [11,42] Phagocytosis Normal phagocytosis and killing of encapsulated bacteria [10]; nominally affected opsonification of yeast particles [11]; select opsonic defect against Pseudomonas [51] not screened for or identified in our retrospective practice database analysis. These cases are typically found dur...…”

“…In children, infectious agents have included Pneumocystis carinii [9], Giardia [10], Staphylococcus [10,11], Salmonella [12], Listeria monocytogenes [13], meningococcus [6,14,15], Pseudomonas [10,16], molluscum contagiosum [17], cytomegalovirus [18], and varicella [17]. These organisms account for recurrent infectious dermatitis, diarrhea, meningitis, upper and lower respiratory infections, sepsis, and in some cases, death.…”

DiGeorge syndrome with hypogammaglobulinaemia: a patient with excess suppressor T cell activity treated with fetal thymus transplantation

“…Surface marker analyses of peripheral blood MNC were performed by direct and indirect immunofluorescence with affinity-purified goat antibodies against human F(ab')2, g, 8, 3' or a chains coupled with fluorescein isothiocyanate (FITC) [13] and OKT3, OKT4, OKT8, OKT10, OKTll, OKIal and OKM1 (anti-monocyte/ granulocyte) monoclonal antibodies (Ortho Diagnostic Systems Inc., Raritan, NJ, USA) and FITC-coupled goat antimouse Ig antibodies (Tago Inc., Burlingame, Calif., USA) as second antibodies [14].…”

Selective Immunoglobulin M Deficiency Presenting with Recurrent Impetigo: A Case Report and Review of the Literature

“…Despite normal quantitative IgG in most patients, IgG antibody responses to diphtheria-tetanus and/or pneumococcal polysaccharide antigens were generally found to be depressed [11]. No phagocytic or complement abnormalities were found, and elevated serum IgE levels and in vivo impairment of antibody production were shown in some patients [12, 13]. Cell-mediated immunity appears to be intact, but the number of detailed studies to confirm this is insufficient [5].…”