U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Combined immunodeficiency with skin granulomas(CCHIDG)

MedGen UID:
435945
Concept ID:
C2673536
Disease or Syndrome
Synonym: Combined cellular and humoral immune defects with granulomas
 
Genes (locations): RAG1 (11p12); RAG2 (11p12)
 
Monarch Initiative: MONDO:0009306
OMIM®: 233650
Orphanet: ORPHA157949

Definition

A rare, genetic, non-severe combined immunodeficiency disease characterized by immunodeficiency (manifested by recurrent and/or severe bacterial and viral infections), destructive noninfectious granulomas involving skin, mucosa and internal organs, and various autoimmune manifestations (including cytopenias, vitiligo, psoriasis, myasthenia gravis, enteropathy). Immunophenotypically, T-cell and B-cell lymphopenia, hypogammaglobulinemia, abnormal specific antibody production and impaired T-cell function are observed. [from ORDO]

Clinical features

From HPO
Recurrent respiratory infections
MedGen UID:
812812
Concept ID:
C3806482
Finding
An increased susceptibility to respiratory infections as manifested by a history of recurrent respiratory infections.
B lymphocytopenia
MedGen UID:
340780
Concept ID:
C1855067
Finding
An abnormal decrease from the normal count of B cells.
Combined immunodeficiency
MedGen UID:
751396
Concept ID:
C2711630
Disease or Syndrome
A group of phenotypically heterogeneous genetic disorders characterized by profound deficiencies of T- and B-cell function, which predispose the patients to both infectious and noninfectious complications.
T lymphocytopenia
MedGen UID:
419385
Concept ID:
C2931322
Finding
An abnormally low count of T cells.
Decreased circulating IgG concentration
MedGen UID:
1720114
Concept ID:
C5234937
Finding
An abnormally decreased level of immunoglobulin G (IgG) in blood.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCombined immunodeficiency with skin granulomas
Follow this link to review classifications for Combined immunodeficiency with skin granulomas in Orphanet.

Recent clinical studies

Etiology

Groß M, Speckmann C, May A, Gajardo-Carrasco T, Wustrau K, Maier SL, Panning M, Huzly D, Agaimy A, Bryceson YT, Choo S, Chow CW, Dückers G, Fasth A, Fraitag S, Gräwe K, Haxelmans S, Holzinger D, Hudowenz O, Hübschen JM, Khurana C, Kienle K, Klifa R, Korn K, Kutzner H, Lämmermann T, Ledig S, Lipsker D, Meeths M, Naumann-Bartsch N, Rascon J, Schänzer A, Seidl M, Tesi B, Vauloup-Fellous C, Vollmer-Kary B, Warnatz K, Wehr C, Neven B, Vargas P, Sepulveda FE, Lehmberg K, Schmitt-Graeff A, Ehl S
J Allergy Clin Immunol 2022 Jan;149(1):388-399.e4. Epub 2021 May 24 doi: 10.1016/j.jaci.2021.05.007. PMID: 34033843
Perelygina L, Faisthalab R, Abernathy E, Chen MH, Hao L, Bercovitch L, Bayer DK, Noroski LM, Lam MT, Cicalese MP, Al-Herz W, Nanda A, Hajjar J, Vanden Driessche K, Schroven S, Leysen J, Rosenbach M, Peters P, Raedler J, Albert MH, Abraham RS, Rangarjan HG, Buchbinder D, Kobrynski L, Pham-Huy A, Dhossche J, Cunningham Rundles C, Meyer AK, Theos A, Atkinson TP, Musiek A, Adeli M, Derichs U, Walz C, Krüger R, von Bernuth H, Klein C, Icenogle J, Hauck F, Sullivan KE
Front Immunol 2021;12:796065. Epub 2021 Dec 20 doi: 10.3389/fimmu.2021.796065. PMID: 35003119Free PMC Article
Al-Herz W, Zainal M, Nanda A
Front Immunol 2021;12:751469. Epub 2021 Sep 30 doi: 10.3389/fimmu.2021.751469. PMID: 34659256Free PMC Article
Harp J, Coggshall K, Ruben BS, Ramírez-Valle F, He SY, Berger TG
Int J Dermatol 2015 Jun;54(6):617-25. Epub 2015 Mar 13 doi: 10.1111/ijd.12765. PMID: 25773292
Deps P, Lucas S, Porro AM, Maeda SM, Tomimori J, Guidella C, Reuter T, Oliveira NS, Madureira BP, Souza VA, Loureiro RM, Alves BL, Bellone A, Lockwood DN
Clin Exp Dermatol 2013 Jul;38(5):470-7. Epub 2013 May 17 doi: 10.1111/ced.12028. PMID: 23678890

Diagnosis

Kyriakou G, Gialeli E, Vryzaki E, Koumoundourou D, Glentis A, Georgiou S
Dermatol Online J 2019 Jun 15;25(6) PMID: 31329385
Leclerc-Mercier S, Moshous D, Neven B, Mahlaoui N, Martin L, Pellier I, Blanche S, Picard C, Fischer A, Perot P, Eloit M, Fraitag S, Bodemer C
J Eur Acad Dermatol Venereol 2019 Jul;33(7):1412-1420. Epub 2019 Apr 15 doi: 10.1111/jdv.15568. PMID: 30869812
Najem CE, Springer J, Prayson R, Culver DA, Fernandez J, Tavee J, Hajj-Ali RA
Semin Arthritis Rheum 2018 Jun;47(6):890-896. Epub 2017 Oct 20 doi: 10.1016/j.semarthrit.2017.10.014. PMID: 29277461
Sillevis Smitt JH, Kuijpers TW
Curr Opin Pediatr 2013 Aug;25(4):492-7. doi: 10.1097/MOP.0b013e3283623b9f. PMID: 23744097
Gadola SD, Moins-Teisserenc HT, Trowsdale J, Gross WL, Cerundolo V
Clin Exp Immunol 2000 Aug;121(2):173-8. doi: 10.1046/j.1365-2249.2000.01264.x. PMID: 10931128Free PMC Article

Therapy

Groß M, Speckmann C, May A, Gajardo-Carrasco T, Wustrau K, Maier SL, Panning M, Huzly D, Agaimy A, Bryceson YT, Choo S, Chow CW, Dückers G, Fasth A, Fraitag S, Gräwe K, Haxelmans S, Holzinger D, Hudowenz O, Hübschen JM, Khurana C, Kienle K, Klifa R, Korn K, Kutzner H, Lämmermann T, Ledig S, Lipsker D, Meeths M, Naumann-Bartsch N, Rascon J, Schänzer A, Seidl M, Tesi B, Vauloup-Fellous C, Vollmer-Kary B, Warnatz K, Wehr C, Neven B, Vargas P, Sepulveda FE, Lehmberg K, Schmitt-Graeff A, Ehl S
J Allergy Clin Immunol 2022 Jan;149(1):388-399.e4. Epub 2021 May 24 doi: 10.1016/j.jaci.2021.05.007. PMID: 34033843
Kyriakou G, Gialeli E, Vryzaki E, Koumoundourou D, Glentis A, Georgiou S
Dermatol Online J 2019 Jun 15;25(6) PMID: 31329385
Farmer JR, Foldvari Z, Ujhazi B, De Ravin SS, Chen K, Bleesing JJH, Schuetz C, Al-Herz W, Abraham RS, Joshi AY, Costa-Carvalho BT, Buchbinder D, Booth C, Reiff A, Ferguson PJ, Aghamohammadi A, Abolhassani H, Puck JM, Adeli M, Cancrini C, Palma P, Bertaina A, Locatelli F, Di Matteo G, Geha RS, Kanariou MG, Lycopoulou L, Tzanoudaki M, Sleasman JW, Parikh S, Pinero G, Fischer BM, Dbaibo G, Unal E, Patiroglu T, Karakukcu M, Al-Saad KK, Dilley MA, Pai SY, Dutmer CM, Gelfand EW, Geier CB, Eibl MM, Wolf HM, Henderson LA, Hazen MM, Bonfim C, Wolska-Kuśnierz B, Butte MJ, Hernandez JD, Nicholas SK, Stepensky P, Chandrakasan S, Miano M, Westermann-Clark E, Goda V, Kriván G, Holland SM, Fadugba O, Henrickson SE, Ozen A, Karakoc-Aydiner E, Baris S, Kiykim A, Bredius R, Hoeger B, Boztug K, Pashchenko O, Neven B, Moshous D, Villartay JP, Bousfiha AA, Hill HR, Notarangelo LD, Walter JE
J Allergy Clin Immunol Pract 2019 Jul-Aug;7(6):1970-1985.e4. Epub 2019 Mar 12 doi: 10.1016/j.jaip.2019.02.038. PMID: 30877075Free PMC Article
Esenboga S, Akal C, Karaatmaca B, Erman B, Dogan S, Orhan D, Boztug K, Ayvaz D, Tezcan İ
Clin Immunol 2018 Dec;197:1-5. Epub 2018 Aug 16 doi: 10.1016/j.clim.2018.08.002. PMID: 30121298
Gadola SD, Moins-Teisserenc HT, Trowsdale J, Gross WL, Cerundolo V
Clin Exp Immunol 2000 Aug;121(2):173-8. doi: 10.1046/j.1365-2249.2000.01264.x. PMID: 10931128Free PMC Article

Prognosis

Geier CB, Farmer JR, Foldvari Z, Ujhazi B, Steininger J, Sleasman JW, Parikh S, Dilley MA, Pai SY, Henderson L, Hazen M, Neven B, Moshous D, Sharapova SO, Mihailova S, Yankova P, Naumova E, Özen S, Byram K, Fernandez J, Wolf HM, Eibl MM, Notarangelo LD, Calabrese LH, Walter JE
Front Immunol 2020;11:574738. Epub 2020 Oct 21 doi: 10.3389/fimmu.2020.574738. PMID: 33193364Free PMC Article
Kyriakou G, Gialeli E, Vryzaki E, Koumoundourou D, Glentis A, Georgiou S
Dermatol Online J 2019 Jun 15;25(6) PMID: 31329385
Mitra A, Pollock B, Gooi J, Darling JC, Boon A, Newton-Bishop JA
Br J Dermatol 2005 Jul;153(1):194-9. doi: 10.1111/j.1365-2133.2005.06619.x. PMID: 16029350
Mehrany K, Kist JM, Gibson LE
Int J Dermatol 2002 Nov;41(11):773-4. doi: 10.1046/j.1365-4362.2002.01542.x. PMID: 12453001
Fischl MA, Daikos GL, Uttamchandani RB, Poblete RB, Moreno JN, Reyes RR, Boota AM, Thompson LM, Cleary TJ, Oldham SA
Ann Intern Med 1992 Aug 1;117(3):184-90. doi: 10.7326/0003-4819-117-3-184. PMID: 1616212

Clinical prediction guides

Darazam IA, Hakamifard A, Momenilandi M, Materna M, Gharehbagh FJ, Shahrooei M, Olyaei NA, Zerehpoosh FB, Fayand A, Hatami F, Lotfollahi L, Mansouri N, Casanova JL, Béziat V, Mansouri D
J Clin Immunol 2023 Jan;43(1):217-228. Epub 2022 Oct 13 doi: 10.1007/s10875-022-01374-7. PMID: 36227411
Leclerc-Mercier S, Moshous D, Neven B, Mahlaoui N, Martin L, Pellier I, Blanche S, Picard C, Fischer A, Perot P, Eloit M, Fraitag S, Bodemer C
J Eur Acad Dermatol Venereol 2019 Jul;33(7):1412-1420. Epub 2019 Apr 15 doi: 10.1111/jdv.15568. PMID: 30869812
Patiroglu T, Akar HH, Van Der Burg M
Acta Microbiol Immunol Hung 2015 Dec;62(4):393-401. doi: 10.1556/030.62.2015.4.4. PMID: 26689875
Nanda A, Al-Herz W, Al-Sabah H, Al-Ajmi H
Am J Dermatopathol 2014 Oct;36(10):832-7. doi: 10.1097/DAD.0000000000000112. PMID: 25062261
Moshous D, Meyts I, Fraitag S, Janssen CE, Debré M, Suarez F, Toelen J, De Boeck K, Roskams T, Deschildre A, Picard C, Bodemer C, Wouters C, Fischer A
J Allergy Clin Immunol 2011 Oct;128(4):847-53. Epub 2011 Jun 28 doi: 10.1016/j.jaci.2011.05.024. PMID: 21714993

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...