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Severe varicella zoster infection

MedGen UID:
1688785
Concept ID:
C5139168
Disease or Syndrome
HPO: HP:0032170

Definition

An unusually severe form of varicella zoster virus (VZV) infection. In the majority of the cases, especially in children, varicella is a very mild infection characterised by skin lesions, low grade fever and malaise. Severe infection is characterized by manifestions including VZV pneumonia, hepatitis, meningitis, and disseminated varicella. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSevere varicella zoster infection

Conditions with this feature

Immunodeficiency due to CD25 deficiency
MedGen UID:
377894
Concept ID:
C1853392
Disease or Syndrome
Immunodeficiency-41 is an autosomal recessive complex disorder of immune dysregulation. Affected individuals present in infancy with recurrent viral, fungal, and bacterial infections, lymphadenopathy, and variable autoimmune features, such as autoimmune enteropathy and eczematous skin lesions. Immunologic studies show a defect in T-cell regulation (summary by Goudy et al., 2013).
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
MedGen UID:
477076
Concept ID:
C3275445
Disease or Syndrome
XMEN is an X-linked recessive immunodeficiency characterized by CD4 (186940) lymphopenia, severe chronic viral infections, and defective T-lymphocyte activation (Li et al., 2011). Affected individuals have chronic Epstein-Barr virus (EBV) infection and are susceptible to the development of EBV-associated B-cell lymphoproliferative disorders. Magnesium supplementation may be therapeutic (summary by Li et al., 2014).
Lymphoproliferative syndrome 2
MedGen UID:
767454
Concept ID:
C3554540
Disease or Syndrome
Lymphoproliferative syndrome-2, also known as CD27 deficiency, is an autosomal recessive immunodeficiency disorder associated with persistent symptomatic EBV viremia, hypogammaglobulinemia, and impairment in specific antibody function resulting from impaired T cell-dependent B-cell responses and T-cell dysfunction (summary by van Montfrans et al., 2012). The phenotype can vary significantly, from asymptomatic borderline-low hypogammaglobulinemia, to a full-blown symptomatic systemic inflammatory response with life-threatening EBV-related complications, including hemophagocytic lymphohistiocytosis, a lymphoproliferative disorder, and malignant lymphoma requiring stem cell transplantation (summary by Salzer et al., 2013). For a discussion of genetic heterogeneity of lymphoproliferative syndrome, see XLP1 (308240).
TCR-alpha-beta-positive T-cell deficiency
MedGen UID:
815662
Concept ID:
C3809332
Disease or Syndrome
Immunodeficiency-7 (IMD7) is an autosomal recessive immunologic disorder characterized by onset of recurrent bacterial and viral infections in infancy or early childhood. Affected individuals may also have features of immune dysregulation, including lymphadenopathy and presence of autoantibodies. Laboratory studies show increased serum IgE, low numbers of T cells, low TCR-alpha/beta cells, and increased TCR-gamma/delta cells. The disorder often results in death in childhood, although bone marrow transplant is effective (summary by Morgan et al., 2011 and Rawat et al., 2021).
Autosomal recessive primary immunodeficiency with defective spontaneous natural killer cell cytotoxicity
MedGen UID:
816672
Concept ID:
C3810342
Disease or Syndrome
Immunodeficiency-20 is a rare autosomal recessive primary immunodeficiency characterized by functional deficiency of NK cells. Patient NK cells are defective in spontaneous cell cytotoxicity, but retain antibody-dependent cellular cytotoxicity. Patients typically present early in childhood with severe herpes viral infections, particularly Epstein Barr virus (EBV), and human papillomavirus (HPV) (summary by Grier et al., 2012).
Immunodeficiency 23
MedGen UID:
862808
Concept ID:
C4014371
Disease or Syndrome
IMD23 is an autosomal recessive primary immunodeficiency syndrome characterized by onset of recurrent infections, usually respiratory or cutaneous, in early childhood. Immune workup usually shows neutropenia, lymphopenia, eosinophilia, and increased serum IgE or IgA. Neutrophil chemotactic defects have also been reported. Infectious agents include bacteria, viruses, and fungi. Many patients develop atopic dermatitis, eczema, and other signs of autoinflammation. Affected individuals may also show developmental delay or cognitive impairment of varying severity (summary by Bjorksten and Lundmark, 1976 and Zhang et al., 2014).
Severe combined immunodeficiency due to CTPS1 deficiency
MedGen UID:
863054
Concept ID:
C4014617
Disease or Syndrome
IMD24 is an autosomal recessive immunodeficiency characterized by the impaired capacity of activated T and B cells to proliferate in response to antigen receptor-mediated activation. Patients have early onset of severe chronic viral infections, mostly caused by herpesviruses, including Epstein-Barr virus (EBV) and varicella zoster virus (VZV); they also suffer from recurrent encapsulated bacterial infections, a spectrum typical of a combined deficiency of adaptive immunity (CID) (summary by Martin et al., 2014).
DOCK2 deficiency
MedGen UID:
901370
Concept ID:
C4225328
Disease or Syndrome
Immunodeficiency-40 is an autosomal recessive primary form of combined immunodeficiency mainly affecting T-cell number and function, with other more variable defects in B-cell and NK-cell function. Patients have onset of severe invasive bacterial and viral infections in early childhood and may die without bone marrow transplantation (summary by Dobbs et al., 2015).
Inflammatory bowel disease, immunodeficiency, and encephalopathy
MedGen UID:
1648434
Concept ID:
C4748708
Disease or Syndrome
A rare genetic disease characterized by infantile onset of severe inflammatory bowel disease manifesting with bloody diarrhea and failure to thrive, and central nervous system disease with global developmental delay and regression, impaired speech, hypotonia, hyperreflexia, and epilepsy. Brain imaging shows global cerebral atrophy, thin corpus callosum, delayed myelination, and posterior leukoencephalopathy. Cases with recurrent infections and impaired T-cell responses to stimulation, as well as decreased T-cell subsets, have been reported.
Immunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
MedGen UID:
1740566
Concept ID:
C5436549
Disease or Syndrome
Immunodeficiency-73B with defective neutrophil chemotaxis (IMD73B) is an autosomal dominant immunologic disorder characterized by onset of recurrent infections in infancy or early childhood. Affected individuals develop respiratory infections, cellulitis, and severe invasive infections or sepsis; organisms include bacteria such as Staphylococcus, as well as viruses, fungi, and mycobacterial species. Laboratory studies show variable abnormalities, including B- and T-cell lymphopenia, decreased immunoglobulin subsets, decreased TRECs and dysfunctional T cells, decreased NK cells, neutropenia, and impaired neutrophil chemotaxis. Hematopoietic stem cell transplantation is curative (summary by Hsu et al., 2019; review by Lougaris et al., 2020). In a review of autosomal forms of chronic granulomatous disease (see 306400 for genetic heterogeneity of CGD), Roos et al. (2021) noted that patients with RAC2 mutations may manifest CGD-like symptoms due to defects in neutrophil NADPH oxidase activity.
Severe combined immunodeficiency due to CD70 deficiency
MedGen UID:
1799982
Concept ID:
C5568559
Disease or Syndrome
Lymphoproliferative syndrome-3 (LPFS3) is an autosomal recessive early-onset immunologic disorder characterized by increased susceptibility to Epstein-Barr virus (EBV) infection in B cells, resulting in abnormal B-cell proliferation and increased susceptibility to B-cell malignancies, including Hodgkin lymphoma. Patients usually have hypogammaglobulinemia without lymphopenia, although some subsets of immune cells may be low and some patients may have recurrent infections. The disorder results from impaired signaling from proliferating B cells to effector T cells that provide immune surveillance. There may be an increased risk of solid tumors in heterozygous carriers (summary by Abolhassani et al., 2017). For a discussion of genetic heterogeneity of lymphoproliferative syndrome, see XLP1 (308240).
Immunodeficiency 102
MedGen UID:
1812534
Concept ID:
C5676886
Disease or Syndrome
Immunodeficiency-102 (IMD102) is an X-linked recessive immunologic disorder characterized by the onset of recurrent sinopulmonary, mucosal, and other infections in early childhood, usually accompanied by refractory autoimmune cytopenias. Affected individuals have bacterial, viral, and fungal infections, as well as hemolytic anemia, thrombocytopenia, lymphopenia, and decreased NK cells. Laboratory studies show defective T-cell proliferation and function, likely due to signaling abnormalities. The disorder may also manifest as a hyperinflammatory state with immune dysregulation (Delmonte et al., 2021).

Professional guidelines

PubMed

Patil A, Goldust M, Wollina U
Viruses 2022 Jan 19;14(2) doi: 10.3390/v14020192. PMID: 35215786Free PMC Article
Heckmann JG, Urban PP, Pitz S, Guntinas-Lichius O, Gágyor I
Dtsch Arztebl Int 2019 Oct 11;116(41):692-702. doi: 10.3238/arztebl.2019.0692. PMID: 31709978Free PMC Article
Koshy E, Mengting L, Kumar H, Jianbo W
Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):251-262. doi: 10.4103/ijdvl.IJDVL_1021_16. PMID: 29516900

Recent clinical studies

Etiology

Patil A, Goldust M, Wollina U
Viruses 2022 Jan 19;14(2) doi: 10.3390/v14020192. PMID: 35215786Free PMC Article
Andrei G, Snoeck R
Molecules 2021 Feb 20;26(4) doi: 10.3390/molecules26041132. PMID: 33672709Free PMC Article
Beaugerie L, Rahier JF, Kirchgesner J
Clin Gastroenterol Hepatol 2020 May;18(6):1324-1335.e2. Epub 2020 Feb 12 doi: 10.1016/j.cgh.2020.02.009. PMID: 32059920
Heckmann JG, Urban PP, Pitz S, Guntinas-Lichius O, Gágyor I
Dtsch Arztebl Int 2019 Oct 11;116(41):692-702. doi: 10.3238/arztebl.2019.0692. PMID: 31709978Free PMC Article
Koshy E, Mengting L, Kumar H, Jianbo W
Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):251-262. doi: 10.4103/ijdvl.IJDVL_1021_16. PMID: 29516900

Diagnosis

Gundamraj V, Hasbun R
Curr Opin Infect Dis 2023 Jun 1;36(3):177-185. Epub 2023 Apr 12 doi: 10.1097/QCO.0000000000000922. PMID: 37093042
Heckmann JG, Urban PP, Pitz S, Guntinas-Lichius O, Gágyor I
Dtsch Arztebl Int 2019 Oct 11;116(41):692-702. doi: 10.3238/arztebl.2019.0692. PMID: 31709978Free PMC Article
Kennedy PGE, Gershon AA
Viruses 2018 Nov 2;10(11) doi: 10.3390/v10110609. PMID: 30400213Free PMC Article
John AR, Canaday DH
Infect Dis Clin North Am 2017 Dec;31(4):811-826. doi: 10.1016/j.idc.2017.07.016. PMID: 29079160Free PMC Article
Gershon AA, Breuer J, Cohen JI, Cohrs RJ, Gershon MD, Gilden D, Grose C, Hambleton S, Kennedy PG, Oxman MN, Seward JF, Yamanishi K
Nat Rev Dis Primers 2015 Jul 2;1:15016. doi: 10.1038/nrdp.2015.16. PMID: 27188665Free PMC Article

Therapy

Patil A, Goldust M, Wollina U
Viruses 2022 Jan 19;14(2) doi: 10.3390/v14020192. PMID: 35215786Free PMC Article
Beaugerie L, Rahier JF, Kirchgesner J
Clin Gastroenterol Hepatol 2020 May;18(6):1324-1335.e2. Epub 2020 Feb 12 doi: 10.1016/j.cgh.2020.02.009. PMID: 32059920
Koshy E, Mengting L, Kumar H, Jianbo W
Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):251-262. doi: 10.4103/ijdvl.IJDVL_1021_16. PMID: 29516900
John AR, Canaday DH
Infect Dis Clin North Am 2017 Dec;31(4):811-826. doi: 10.1016/j.idc.2017.07.016. PMID: 29079160Free PMC Article
Gershon AA, Breuer J, Cohen JI, Cohrs RJ, Gershon MD, Gilden D, Grose C, Hambleton S, Kennedy PG, Oxman MN, Seward JF, Yamanishi K
Nat Rev Dis Primers 2015 Jul 2;1:15016. doi: 10.1038/nrdp.2015.16. PMID: 27188665Free PMC Article

Prognosis

Majewska A, Mlynarczyk-Bonikowska B
Int J Mol Sci 2022 Mar 22;23(7) doi: 10.3390/ijms23073431. PMID: 35408788Free PMC Article
Patil A, Goldust M, Wollina U
Viruses 2022 Jan 19;14(2) doi: 10.3390/v14020192. PMID: 35215786Free PMC Article
Beaugerie L, Rahier JF, Kirchgesner J
Clin Gastroenterol Hepatol 2020 May;18(6):1324-1335.e2. Epub 2020 Feb 12 doi: 10.1016/j.cgh.2020.02.009. PMID: 32059920
Koshy E, Mengting L, Kumar H, Jianbo W
Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):251-262. doi: 10.4103/ijdvl.IJDVL_1021_16. PMID: 29516900
Oxman MN, Levin MJ, Johnson GR, Schmader KE, Straus SE, Gelb LD, Arbeit RD, Simberkoff MS, Gershon AA, Davis LE, Weinberg A, Boardman KD, Williams HM, Zhang JH, Peduzzi PN, Beisel CE, Morrison VA, Guatelli JC, Brooks PA, Kauffman CA, Pachucki CT, Neuzil KM, Betts RF, Wright PF, Griffin MR, Brunell P, Soto NE, Marques AR, Keay SK, Goodman RP, Cotton DJ, Gnann JW Jr, Loutit J, Holodniy M, Keitel WA, Crawford GE, Yeh SS, Lobo Z, Toney JF, Greenberg RN, Keller PM, Harbecke R, Hayward AR, Irwin MR, Kyriakides TC, Chan CY, Chan IS, Wang WW, Annunziato PW, Silber JL; Shingles Prevention Study Group
N Engl J Med 2005 Jun 2;352(22):2271-84. doi: 10.1056/NEJMoa051016. PMID: 15930418

Clinical prediction guides

Loftus EV Jr, Panés J, Lacerda AP, Peyrin-Biroulet L, D'Haens G, Panaccione R, Reinisch W, Louis E, Chen M, Nakase H, Begun J, Boland BS, Phillips C, Mohamed MF, Liu J, Geng Z, Feng T, Dubcenco E, Colombel JF
N Engl J Med 2023 May 25;388(21):1966-1980. doi: 10.1056/NEJMoa2212728. PMID: 37224198
Patil A, Goldust M, Wollina U
Viruses 2022 Jan 19;14(2) doi: 10.3390/v14020192. PMID: 35215786Free PMC Article
Auriti C, De Rose DU, Santisi A, Martini L, Piersigilli F, Bersani I, Ronchetti MP, Caforio L
Biochim Biophys Acta Mol Basis Dis 2021 Oct 1;1867(10):166198. Epub 2021 Jun 10 doi: 10.1016/j.bbadis.2021.166198. PMID: 34118406Free PMC Article
López-Sanromán A, Esplugues JV, Domènech E
Gastroenterol Hepatol 2021 Jan;44(1):39-48. Epub 2020 Aug 20 doi: 10.1016/j.gastrohep.2020.04.012. PMID: 32829958
Beaugerie L, Rahier JF, Kirchgesner J
Clin Gastroenterol Hepatol 2020 May;18(6):1324-1335.e2. Epub 2020 Feb 12 doi: 10.1016/j.cgh.2020.02.009. PMID: 32059920

Recent systematic reviews

Alves C, Penedones A, Mendes D, Batel Marques F
Eur J Clin Pharmacol 2022 Dec;78(12):1923-1933. Epub 2022 Oct 7 doi: 10.1007/s00228-022-03400-4. PMID: 36207461
Di Pietrantonj C, Rivetti A, Marchione P, Debalini MG, Demicheli V
Cochrane Database Syst Rev 2021 Nov 22;11(11):CD004407. doi: 10.1002/14651858.CD004407.pub5. PMID: 34806766Free PMC Article
McKay SL, Guo A, Pergam SA, Dooling K
Clin Infect Dis 2020 Oct 23;71(7):e125-e134. doi: 10.1093/cid/ciz1090. PMID: 31677266Free PMC Article
Forbes HJ, Thomas SL, Smeeth L, Clayton T, Farmer R, Bhaskaran K, Langan SM
Pain 2016 Jan;157(1):30-54. doi: 10.1097/j.pain.0000000000000307. PMID: 26218719Free PMC Article
Cohen J, Breuer J
BMJ Clin Evid 2015 Jun 15;2015 PMID: 26077272Free PMC Article

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