Extensive Molluscum Contagiosum in X-Linked Agammaglobulinemia

X-linked agammaglobulinemia (XLA) is among the most common inborn errors of immunity. The usual clinical phenotype an increased susceptibility to bacterial and enteroviral infections; however, other viral infections, which result in chronic, severe, or recurrent are being increasingly reported XLA. These include respiratory viruses, herpesviruses, hepatotropic more rarely members from astrovirus orthobunyavirus families.1Jones T.P.W. Buckland M. Breuer J. Lowe D.M. Viral infection primary antibody deficiency syndromes.Rev Med Virol. 2019; 29: e2049Crossref PubMed Scopus (16) Google Scholar Molluscum contagiosum, a poxvirus, has not been patients with A 4.5-year-old boy presented history sinopulmonary infections since age 4 months. episodes were managed elsewhere using oral intravenous antimicrobials. On examination, his weight height less than −3 SD. Multiple pearly molluscum contagiosum lesions noted over chin (arrow), rest face, neck, ears, hands (Figure 1). He also had cellulitis on right cheek (arrowhead). Laboratory investigations showed normal total leukocyte count (11.2 × 109/L), differential counts (neutrophils, 38%; lymphocytes, 50%; monocytes, 9%; eosinophils 3%), undetectable serum IgG, IgA, IgE levels. Flow cytometry CD3+ T cells 86.13% (N = 56-75), CD4+ 41.62% 28-47), CD8+ 41.08% 16-30), CD19+ B 0.01% 14-33), CD56+ natural killer 8.32% 4-17). Naive helper lymphocytes (CD45RA+ CD4+) (56.81% [N 53-86]), TH17 (CD4+ IL-17+) reduced (0.2%). Targeted next-generation sequencing nonsense mutation BTK gene (c.752G>A, p.Trp251Ter). patient was diagnosed have XLA initiated replacement immunoglobulin therapy. Cellulitis treated amoxicillin, topical trichloroacetic acid application. one humoral immunodeficiency. Although considered be pure B- cell deficiency, T-cell dysfunction these patients. Edwards et al2Edwards E.S.J. Bosco J.J. Aui P.M. Stirling R.G. Cameron P.U. Chatelier al.Predominantly antibody-deficient non-infectious complications naive B, Treg, Th17, Tfh17 Cells.Front Immunol. 10: 2593Crossref (18) low numbers follicular 17 This could help explain increasing spectrum seen index found cells; we perform functional assays. may children. However, severe and/or suggestive findings need evaluation for secondary immunodeficiency diseases. Children HIV infection, especially CD4 counts, develop extensive contagiosum; case negative elevated absolute count. Several combined cellular immunity associated predisposition infections. Wiskott-Aldrich syndrome, warts hypogammaglobulinemia myelokathexis DOCK8 CARMIL2 (RLTPR) STK4 STAT1 gain function, NEMO RHOH polymerase D GATA2 hypomorphic forms Extensive extremely unusual. it difficult conclude basis single report that children predisposed would require data. Cidofovir effective against virus used refractory treatment.