Skip to main content

Recurrent Cold Suppurative Granulomatous Lymphadenitis

  • Chapter
  • First Online:
Pediatric Immunology
  • 46 Accesses

Abstract

Cold abscesses are a hallmark of defects of the innate immune system including hyper-IgE syndrome and chronic granulomatous disease

MyD88 is a molecular adaptor acting downstream to all TLR, except TLR3, and downstream to IL-1R

MyD88 deficiency is associated with selective increased susceptibility to invasive pyogenic bacterial infections

Low fever and mild increase of systemic inflammatory markers in contrast with a severe clinical course of infectious diseases should rise the suspicion of MyD88 deficiency

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Johnston SL. Clinical immunology review series: an approach to the patient with recurrent superficial abscesses. Clin Exp Immunol. 2008;152(3):397–405.

    Article  CAS  Google Scholar 

  2. Joshi N, Caputo GM, Weitekamp MR, Karchmer AW. Infections in patients with diabetes mellitus. N Engl J Med. 1999;341(25):1906–12.

    Article  CAS  Google Scholar 

  3. Ephgrave K. Extra-intestinal manifestations of Crohn’s disease. Surg Clin North Am. 2007;87(3):673–80.

    Article  Google Scholar 

  4. Giardino G, Cicalese MP, Delmonte O, Migliavacca M, Palterer B, Loffredo L, Cirillo E, Gallo V, Violi F, Pignata C. NADPH Oxidase deficiency: a multisystem approach. Oxidative Med Cell Longev. 2017;2017:23.

    Article  Google Scholar 

  5. Dinauer MC. Primary immune deficiencies with defects in neutrophil function. Hematology Am Soc Hematol Educ Program. 2016;2016(1):43–50.

    Article  Google Scholar 

  6. Moin A, Farhoudi A, Moin M, Pourpak Z, Bazargan N. Cutaneous manifestations of primary immunodeficiency diseases in children. Iran J Allergy Asthma Immunol. 2006;5(3):121–6.

    PubMed  Google Scholar 

  7. Reda SM, El-Ghoneimy DH, Afifi HM. Clinical predictors of primary immunodeficiency diseases in children. Allergy, Asthma Immunol Res. 2013;5(2):88–95.

    Article  Google Scholar 

  8. Grimbacher B, Holland SM, Gallin JI, Greenberg F, Hill SC, Malech HL, Miller JA, O'Connell AC, Puck JM. Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder. N Engl J Med. 1999;340(9):692–702.

    Article  CAS  Google Scholar 

  9. Marciano BE, Zerbe CS, Falcone EL, Ding L, DeRavin SS, Daub J, Kreuzburg S, Yockey L, Hunsberger S, Foruraghi L, Barnhart LA, Matharu K, Anderson V, Darnell DN, Frein C, Fink DL, Lau KP, Long Priel DA, Gallin JI, Malech HL, Uzel G, Freeman AF, Kuhns DB, Rosenzweig SD, Holland SM. X-linked carriers of chronic granulomatous disease: illness, lyonization, and stability. J Allergy Clin Immunol. 2018;141(1):365–37.

    Article  CAS  Google Scholar 

  10. Conway DH, Dara J, Bagashev A, Sullivan KE. Myeloid differentiation primary response gene 88 (MyD88) deficiency in a large kindred. J Allergy Clin Immunol. 2010;126(1):172–5.

    Article  CAS  Google Scholar 

  11. Picard C, Casanova JL, Puel A. Infectious diseases in patients with IRAK-4, MyD88, NEMO, or IkappaBalpha deficiency. Clin Microbiol Rev. 2011;24(3):490–7.

    Article  CAS  Google Scholar 

  12. Picard C, von Bernuth H, Ghandil P, Chrabieh M, Levy O, Arkwright PD, McDonald D, Geha RS, Takada H, Krause JC, Creech CB, Ku CL, Ehl S, Marodi L, Al-Muhsen S, Al-Hajjar S, Al-Ghonaium A, Day-Good NK, Holland SM, Gallin JI, Chapel H, Speert DP, Rodriguez-Gallego C, Colino E, Garty BZ, Roifman C, Hara T, Yoshikawa H, Nonoyama S, Domachowske J, Issekutz AC, Tang M, Smart J, Zitnik SE, Hoarau C, Kumararatne DS, Thrasher AJ, Davies EG, Bethune C, Sirvent N, de Ricaud D, Camcioglu Y, Vasconcelos J, Guedes M, Vitor AB, Rodrigo C, Almazan F, Mendez M, Arostegui JI, Alsina L, Fortuny C, Reichenbach J, Verbsky JW, Bossuyt X, Doffinger R, Abel L, Puel A, Casanova JL. Clinical features and outcome of patients with IRAK-4 and MyD88 deficiency. Medicine. 2010;89(6):403–25.

    Article  CAS  Google Scholar 

  13. von Bernuth H, Picard C, Jin Z, Pankla R, Xiao H, Ku CL, Chrabieh M, Mustapha IB, Ghandil P, Camcioglu Y, Vasconcelos J, Sirvent N, Guedes M, Vitor AB, Herrero-Mata MJ, Arostegui JI, Rodrigo C, Alsina L, Ruiz-Ortiz E, Juan M, Fortuny C, Yague J, Anton J, Pascal M, Chang HH, Janniere L, Rose Y, Garty BZ, Chapel H, Issekutz A, Marodi L, Rodriguez-Gallego C, Banchereau J, Abel L, Li X, Chaussabel D, Puel A, Casanova JL. Pyogenic bacterial infections in humans with MyD88 deficiency. Science (New York, NY). 2008;321(5889):691–6.

    Article  Google Scholar 

  14. Giardino G, Gallo V, Somma D, Farrow EG, Thiffault I, D’Assante R, Donofrio V, Paciolla M, Ursini MV, Leonardi A, Saunders CJ, Pignata C. Targeted next-generation sequencing revealed MYD88 deficiency in a child with chronic yersiniosis and granulomatous lymphadenitis. J Allergy Clin Immunol. 2016;137(5):1591–5.e4.

    Article  CAS  Google Scholar 

  15. von Bernuth H, Picard C, Puel A, Casanova JL. Experimental and natural infections in MyD88- and IRAK-4-deficient mice and humans. Eur J Immunol. 2012;42(12):3126–35.

    Article  Google Scholar 

  16. Troutman TD, Bazan JF, Pasare C. Toll-like receptors, signaling adapters and regulation of the pro-inflammatory response by PI3K. Cell Cycle. 2012;11(19):3559–67.

    Article  CAS  Google Scholar 

  17. Bouma G, Doffinger R, Patel SY, Peskett E, Sinclair JC, Barcenas-Morales G, Cerron-Gutierrez L, Kumararatne DS, Davies EG, Thrasher AJ, Burns SO. Impaired neutrophil migration and phagocytosis in IRAK-4 deficiency. Br J Haematol. 2009;147(1):153–6.

    Article  CAS  Google Scholar 

  18. Weller S, Bonnet M, Delagreverie H, Israel L, Chrabieh M, Marodi L, Rodriguez-Gallego C, Garty BZ, Roifman C, Issekutz AC, Zitnik SE, Hoarau C, Camcioglu Y, Vasconcelos J, Rodrigo C, Arkwright PD, Cerutti A, Meffre E, Zhang SY, Alcais A, Puel A, Casanova JL, Picard C, Weill JC, Reynaud CA. IgM+IgD+CD27+ B cells are markedly reduced in IRAK-4-, MyD88-, and TIRAP- but not UNC-93B-deficient patients. Blood. 2012;120(25):4992–5001.

    Article  CAS  Google Scholar 

  19. Maglione PJ, Simchoni N, Black S, Radigan L, Overbey JR, Bagiella E, Bussel JB, Bossuyt X, Casanova JL, Meyts I, Cerutti A, Picard C, Cunningham-Rundles C. IRAK-4 and MyD88 deficiencies impair IgM responses against T-independent bacterial antigens. Blood. 2014;124(24):3561–71.

    Article  CAS  Google Scholar 

  20. Wolpert J, Knutsen AP. Natural history of selective antibody deficiency to bacterial polysaccharide antigens in children. Pediatr Asthma Allergy Immunol. 1998;12(3):183–91.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Claudio Pignata .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Giardino, G., Cirillo, E., Gallo, V., Pignata, C. (2019). Recurrent Cold Suppurative Granulomatous Lymphadenitis. In: Rezaei, N. (eds) Pediatric Immunology. Springer, Cham. https://doi.org/10.1007/978-3-030-21262-9_68

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-21262-9_68

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-21261-2

  • Online ISBN: 978-3-030-21262-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics