Common Variable Immunodeficiency Questions & Answers

1. Boileau J, Mouillot G, Gerard L, et al. Autoimmunity in common variable immunodeficiency: correlation with lymphocyte phenotype in the French DEFI study. J Autoimmun. 2011 Feb. 36(1):25-32. [QxMD MEDLINE Link].

2. Bergler-Czop B, Brzezinska-Wcislo L. Pyoderma gangrenosum in a patient with common variable primary immunodeficiency. Postepy Dermatol Alergol. 2013 Jun. 30(3):188-91. [QxMD MEDLINE Link]. [Full Text].

3. Simsek O, Ulusan K, Orhan A, Kiris T, Kocael A, Kocael P. Pyoderma gangrenosum with common variable immunodeficiency. Wounds. 2015 May. 27(5):129-33. [QxMD MEDLINE Link].

4. Arunachalam M, Sanzo M, Lotti T, Colucci R, Berti S, Moretti S. Common variable immunodeficiency in vitiligo. G Ital Dermatol Venereol. 2010 Dec. 145(6):783-8. [QxMD MEDLINE Link].

5. Alvarez-Cuesta C, Molinos L, Cascante JA, Soler T, Perez-Oliva N. Cutaneous granulomas in a patient with common variable immunodeficiency. Acta Derm Venereol. 1999 Jul. 79(4):334. [QxMD MEDLINE Link].

6. Krupnick AI, Shim H, Phelps RG, Cunningham-Rundles C, Sapadin AN. Cutaneous granulomas masquerading as tuberculoid leprosy in a patient with congenital combined immunodeficiency. Mt Sinai J Med. 2001 Sep-Oct. 68(4-5):326-30. [QxMD MEDLINE Link].

7. Mitra A, Pollock B, Gooi J, Darling JC, Boon A, Newton-Bishop JA. Cutaneous granulomas associated with primary immunodeficiency disorders. Br J Dermatol. 2005 Jul. 153(1):194-9. [QxMD MEDLINE Link].

8. Levine TS, Price AB, Boyle S, Webster AD. Cutaneous sarcoid-like granulomas in primary immunodeficiency disorders. Br J Dermatol. 1994 Jan. 130(1):118-20. [QxMD MEDLINE Link].

9. Pagnini I, Simonini G, Lippi F, Azzari C, Cimaz R. Cutaneous polyarteritis nodosa and common variable immunodeficiency: a previously unreported association. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S169. [QxMD MEDLINE Link].

10. Ma CM, Chen WC, Shen JL, Tseng CW, Lin CT, Yang CS, et al. Cutaneous granulomas in a patient with common variable immunodeficiency disease. J Dtsch Dermatol Ges. 2018 Feb. 16 (2):217-219. [QxMD MEDLINE Link].

11. Abdel-Naser MB, Wollina U, El Hefnawi MA, Habib MA, El Okby M. Non-sarcoidal, non-tuberculoid granuloma in common variable immunodeficiency. J Drugs Dermatol. 2006 Apr. 5(4):370-2. [QxMD MEDLINE Link].

12. Le Saos-Patrinos C, Loizon S, Blanco P, Viallard JF, Duluc D. Functions of Tfh Cells in Common Variable Immunodeficiency. Front Immunol. 2020. 11:6. [QxMD MEDLINE Link].

13. van de Ven AA, van de Corput L, van Tilburg CM, et al. Lymphocyte characteristics in children with common variable immunodeficiency. Clin Immunol. 2009 Dec 11. [QxMD MEDLINE Link].

14. Kienzler AK, Hargreaves CE, Patel SY. The role of genomics in common variable immunodeficiency disorders. Clin Exp Immunol. 2017 Feb 25. [QxMD MEDLINE Link].

15. Christiansen M, Offersen R, Jensen JMB, Petersen MS, Larsen CS, Mogensen TH. Identification of Novel Genetic Variants in CVID Patients With Autoimmunity, Autoinflammation, or Malignancy. Front Immunol. 2019. 10:3022. [QxMD MEDLINE Link].

16. Wong GK, Huissoon AP. T-cell abnormalities in common variable immunodeficiency: the hidden defect. J Clin Pathol. 2016 May 6. [QxMD MEDLINE Link].

17. Cunningham-Rundles C. Autoimmune manifestations in common variable immunodeficiency. J Clin Immunol. 2008 May. 28 Suppl 1:S42-5. [QxMD MEDLINE Link].

18. Rezaei N, Wing J, Aghamohammadi A, et al. B-cell-T-cell activation and interaction in common variable immunodeficiency. Hum Immunol. 2010 Feb 4. [QxMD MEDLINE Link].

19. Abolhassani H, Gharib B, Shahinpour S, Masoom SN, Havaei A, Mirminachi B, et al. Autoimmunity in patients with selective IgA deficiency. J Investig Allergol Clin Immunol. 2015. 25(2):112-9. [QxMD MEDLINE Link].

20. Rezaei N, Aghamohammadi A, Siadat SD, et al. Serum bactericidal antibody responses to meningococcal polysaccharide vaccination as a basis for clinical classification of common variable immunodeficiency. Clin Vaccine Immunol. 2008 Apr. 15(4):607-11. [QxMD MEDLINE Link].

21. Lanio N, Sarmiento E, Gallego A, Carbone J. Immunophenotypic profile of T cells in common variable immunodeficiency: is there an association with different clinical findings?. Allergol Immunopathol (Madr). 2009 Jan-Feb. 37(1):14-20. [QxMD MEDLINE Link].

22. Castigli E, Wilson SA, Garibyan L, et al. TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat Genet. 2005 Aug. 37(8):829-34. [QxMD MEDLINE Link].

23. Schaffer AA, Salzer U, Hammarström L, Grimbacher B. Deconstructing common variable immunodeficiency by genetic analysis. Curr Opin Genet Dev. 2007 Jun. 17(3):201-12. [QxMD MEDLINE Link].

24. Neumann C, Grimbacher B. [Molecular basis of common variable immunodeficiency]. Dtsch Med Wochenschr. 2007 Apr 20. 132(16):885-7. [QxMD MEDLINE Link].

25. Salzer U, Chapel HM, Webster AD, et al. Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans. Nat Genet. 2005 Aug. 37(8):820-8. [QxMD MEDLINE Link].

26. Finck A, Van der Meer JW, Schaffer AA, et al. Linkage of autosomal-dominant common variable immunodeficiency to chromosome 4q. Eur J Hum Genet. 2006 Jul. 14(7):867-75. [QxMD MEDLINE Link].

27. Nijenhuis T, Klasen I, Weemaes CM, Preijers F, de Vries E, van der Meer JW. Common variable immunodeficiency (CVID) in a family: an autosomal dominant mode of inheritance. Neth J Med. 2001 Sep. 59(3):134-9. [QxMD MEDLINE Link].

28. Danieli MG, Mezzanotte C, Verga JU, Menghini D, Pedini V, Bilò MB, et al. Common Variable Immunodeficiency in Elderly Patients: A Long-Term Clinical Experience. Biomedicines. 2022 Mar 9. 10 (3):[QxMD MEDLINE Link]. [Full Text].

29. Chapel H, Lucas M, Lee M, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008 Jul 15. 112(2):277-86. [QxMD MEDLINE Link].

30. Hartono S, Motosue MS, Khan S, Rodriguez V, Iyer VN, Divekar R, et al. Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2017 Feb 18. [QxMD MEDLINE Link].

31. Aghamohammadi A, Farhoudi A, Moin M, et al. Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol. 2005 Jul. 12(7):825-32. [QxMD MEDLINE Link].

32. Kwong JC, Ward PB, Johnson PD. Cutaneous protothecosis in a patient with hypogammaglobulinemia. Med Mycol Case Rep. 2013 Jun 20. 2:132-3. [QxMD MEDLINE Link]. [Full Text].

33. Le Cleach L, Benchikhi H, Liedman D, Boumsel L, Wolkenstein P, Revuz J. [Hand-foot-mouth syndrome recurring during common variable deficiency]. Ann Dermatol Venereol. 1999 Mar. 126(3):251-3. [QxMD MEDLINE Link].

34. Khodadad A, Aghamohammadi A, Parvaneh N, et al. Gastrointestinal manifestations in patients with common variable immunodeficiency. Dig Dis Sci. 2007 Nov. 52(11):2977-83. [QxMD MEDLINE Link].

35. Lassoued K. [Humoral immune deficits and the skin]. Ann Dermatol Venereol. 1999 Mar. 126(3):215-22. [QxMD MEDLINE Link].

36. Boonyaleepun S, Boonyaleepun C, Schlactus JL. Effect of IVIG on the hair regrowth in a common variable immune deficiency patient with alopecia universalis. Asian Pac J Allergy Immunol. 1999 Mar. 17(1):59-62. [QxMD MEDLINE Link].

37. Kilic S, Ersoy F, Sanal O, Turkbay D, Tezcan I. Alopecia universalis in a patient with common variable immunodeficiency. Pediatr Dermatol. 1999 Jul-Aug. 16(4):305-7. [QxMD MEDLINE Link].

38. Sanjuán Álvarez M, Sánchez Zamora P, González Salvador Y, García Rueda A, Herrero Trujillano M, Rodríguez Bertos C. [Necrotising fasciitis in a patient with common variable immunodeficiency.]. Rev Esp Anestesiol Reanim. 2012 May 8. [QxMD MEDLINE Link].

39. Pujol RM, Nadal C, Taberner R, Diaz C, Miralles J, Alomar A. Cutaneous granulomatous lesions in common variable immunodeficiency: complete resolution after intravenous immunoglobulins. Dermatology. 1999. 198(2):156-8. [QxMD MEDLINE Link].

40. Green HA, Moschella S. Multiple invasive squamous cell carcinomas and common variable immunodeficiency. Arch Dermatol. 1992 Mar. 128(3):412-3. [QxMD MEDLINE Link].

41. Creamer D, McGregor JM, Hawk JL. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy. Clin Exp Dermatol. 1999 Jul. 24(4):273-4. [QxMD MEDLINE Link].

42. Modrzewska K, Wiatr E, Langfort R, Oniszh K, Roszkowski-Sliz K. [Common variable immunodeficiency in a patient with suspected sarcoidosis]. Pneumonol Alergol Pol. 2009. 77(1):91-6. [QxMD MEDLINE Link].

43. Filion CA, Taylor-Black S, Maglione PJ, Radigan L, Cunningham-Rundles C. Differentiation of Common Variable Immunodeficiency From IgG Deficiency. J Allergy Clin Immunol Pract. 2019 Apr. 7 (4):1277-1284. [QxMD MEDLINE Link].

44. Lawrence MG, Palacios-Kibler TV, Workman LJ, Schuyler AJ, Steinke JW, Payne SC, et al. Low Serum IgE Is a Sensitive and Specific Marker for Common Variable Immunodeficiency (CVID). J Clin Immunol. 2018 Feb 17. [QxMD MEDLINE Link].

45. Bayrhuber M, Tinsel I, Goldacker S, Kindle G, Warnatz K, Farin E, et al. Perceived health of patients with common variable immunodeficiency - a cluster analysis. Clin Exp Immunol. 2019 Apr. 196 (1):76-85. [QxMD MEDLINE Link].

46. Mullur J, Wang A, Feldweg A. A fatal case of coronavirus disease 2019 in a patient with common variable immunodeficiency. Ann Allergy Asthma Immunol. 2021 Jan. 126 (1):90-92. [QxMD MEDLINE Link].

47. Cohen B, Rubinstein R, Gans MD, Deng L, Rubinstein A, Eisenberg R. COVID-19 infection in 10 common variable immunodeficiency patients in New York City. J Allergy Clin Immunol Pract. 2021 Jan. 9 (1):504-507.e1. [QxMD MEDLINE Link].

48. Quinti I, Di Pietro C, Martini H, Pesce AM, Lombardi F, Baumghartner M, et al. Health related quality of life in common variable immunodeficiency. Yonsei Med J. 2012 May 1. 53(3):603-10. [QxMD MEDLINE Link].

49. Rezaei N, Siadat SD, Aghamohammadi A, et al. Serum Bactericidal Antibody Response One Year after Meningococcal Polysaccharide Vaccination in Patients with Common Variable Immunodeficiency. Clin Vaccine Immunol. 2010 Jan 27. [QxMD MEDLINE Link]. [Full Text].

50. Hanitsch LG, Löbel M, Mieves JF, Bauer S, Babel N, Schweiger B, et al. Cellular and humoral influenza-specific immune response upon vaccination in patients with common variable immunodeficiency and unclassified antibody deficiency. Vaccine. 2016 May 5. 34 (21):2417-23. [QxMD MEDLINE Link].

51. Simonson WT, Markovina S, Grossman WJ, et al. Common variable immunodeficiency with regulatory T-cell deficiency treated with rapamycin. Ann Allergy Asthma Immunol. 2009 Feb. 102(2):170-1. [QxMD MEDLINE Link].

52. Shabbat S, Aharoni J, Sarid L, Ben-Harush M, Kapelushnik J. Rituximab as monotherapy and in addition to reduced CHOP in children with primary immunodeficiency and non-Hodgkin lymphoma. Pediatr Blood Cancer. 2009 May. 52(5):664-6. [QxMD MEDLINE Link].

53. Lin JH, Liebhaber M, Roberts RL, Dyer Z, Stiehm ER. Etanercept treatment of cutaneous granulomas in common variable immunodeficiency. J Allergy Clin Immunol. 2006 Apr. 117(4):878-82. [QxMD MEDLINE Link].

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Rohit M Modak, MD, MBA Staff Physician, Department of Infectious Diseases, Virginia Hospital Center

Disclosure: Nothing to disclose.

Prema Modak, MD Physician, The Eye Center, Inc

Prema Modak, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

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