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Cutaneous anergy

MedGen UID:
344575
Concept ID:
C1855781
Finding
Synonyms: Absence of delayed hypersensitivity skin test; Lack of delayed skin hypersensitivity reaction
 
HPO: HP:0002965

Definition

Inability to react to a delayed hypersensitivity skin test. [from HPO]

Conditions with this feature

T-B+ severe combined immunodeficiency due to JAK3 deficiency
MedGen UID:
331474
Concept ID:
C1833275
Disease or Syndrome
JAK3-deficient severe combined immunodeficiency (SCID) is an inherited disorder of the immune system. Individuals with JAK3-deficient SCID lack the necessary immune cells to fight off certain bacteria, viruses, and fungi. They are prone to repeated and persistent infections that can be very serious or life-threatening. Often the organisms that cause infection in people with JAK3-deficient SCID are described as opportunistic because they ordinarily do not cause illness in healthy people. Affected infants typically develop chronic diarrhea, a fungal infection in the mouth called oral thrush, pneumonia, and skin rashes. Persistent illness also causes affected individuals to grow more slowly than other children. Without treatment, people with JAK3-deficient SCID usually live only into early childhood.
Chronic mucocutaneous candidiasis due to monocyte chemotactic disorder
MedGen UID:
343238
Concept ID:
C1854982
Disease or Syndrome
Vici syndrome
MedGen UID:
340962
Concept ID:
C1855772
Disease or Syndrome
With the current widespread use of multigene panels and comprehensive genomic testing, it has become apparent that the phenotypic spectrum of EPG5-related disorder represents a continuum. At the most severe end of the spectrum is classic Vici syndrome (defined as a neurodevelopmental disorder with multisystem involvement characterized by the combination of agenesis of the corpus callosum, cataracts, hypopigmentation, cardiomyopathy, combined immunodeficiency, microcephaly, and failure to thrive); at the milder end of the spectrum are attenuated neurodevelopmental phenotypes with variable multisystem involvement. Median survival in classic Vici syndrome appears to be 24 months, with only 10% of children surviving longer than age five years; the most common causes of death are respiratory infections as a result of primary immunodeficiency and/or cardiac insufficiency resulting from progressive cardiac failure. No data are available on life span in individuals at the milder end of the spectrum.
Splenomegaly syndrome with splenic Germinal center hypoplasia and reduced circulating T helper cells
MedGen UID:
357126
Concept ID:
C1866744
Disease or Syndrome
Candidiasis, familial, 1
MedGen UID:
414015
Concept ID:
C2751429
Disease or Syndrome
Chronic mucocutaneous candidiasis (CMC) includes a group of rare disorders with altered immune responses, selective against Candida, characterized by persistent and/or recurrent infections of the skin, nails, and mucous membranes, caused by organisms of the genus Candida, mainly Candida albicans (Zuccarello et al., 2002). Isolated familial chronic mucocutaneous candidiasis is distinct from candidiasis with endocrinopathy (240300). In myeloperoxidase deficiency (254600), susceptibility to candidiasis may be increased. Genetic Heterogeneity of Candidiasis Familial candidiasis-1 (CANDF1) maps to chromosome 2p. CANDF2 (212050) is caused by mutation in the CARD9 gene (607212) on chromosome 9q34.3. CANDF3 (607644), a form restricted to nails of the hands and feet, maps to chromosome 11. CANDF4 (613108) is caused by mutation in the CLEC7A gene (606264) on chromosome 12p13. CANDF6 (613956) is caused by mutation in the IL17F gene (606496) on chromosome 6p12. CANDF7 (614162) is caused by mutation in the STAT1 gene (600555) on chromosome 2q32. CANDF8 (615527) is caused by mutation in the TRAF3IP2 gene (607043) on chromosome 6q21. CANDF9 (616445) is caused by mutation in the IL17RC gene (610925) on chromosome 3p25. A form of familial candidiasis, previously thought to be isolated and designated CANDF5, has been found to be part of a primary immune deficiency (IMD51; 613953) that includes Staphylococcal skin infections and increased susceptibility to chronic bacterial respiratory infections.
MHC class II deficiency
MedGen UID:
444051
Concept ID:
C2931418
Disease or Syndrome
A rare autosomal recessive primary immunodeficiency characterized by absence of HLA class II molecules on the surface of immune cells, leading to severely impaired cellular and humoral immune response to foreign antigens, severe CD4+ T-cell lymphopenia, and hypogammaglobulinemia. The disease clinically manifests with early onset of severe and recurrent infections mainly of the respiratory and gastrointestinal tract, protracted diarrhea with failure to thrive, and autoimmune disease, and is frequently fatal in childhood.

Professional guidelines

Recent clinical studies

Etiology

Harris TG, Burk RD, Xue X, Anastos K, Minkoff H, Massad LS, Young MA, Levine AM, Gange SJ, Watts DH, Palefsky JM, Strickler HD
AIDS 2007 Sep 12;21(14):1933-41. doi: 10.1097/QAD.0b013e3282c3a945. PMID: 17721101
Ohrui T
Tohoku J Exp Med 2005 Sep;207(1):3-12. doi: 10.1620/tjem.207.3. PMID: 16082150
MMWR Recomm Rep 1997 Sep 5;46(RR-15):1-10. PMID: 9332982
Lloyd AR, Wakefield D, Hickie I
Ciba Found Symp 1993;173:176-87; discussion 187-92. doi: 10.1002/9780470514382.ch11. PMID: 8491097
Kataria YP, LoBuglio AF, Bromberg PA, Hurtubise PE
Ann N Y Acad Sci 1976;278:69-79. doi: 10.1111/j.1749-6632.1976.tb47017.x. PMID: 786127

Diagnosis

Belfer MH, Stevens RW
Am Fam Physician 1998 Dec;58(9):2041-50, 2055-6. PMID: 9861878
Kerdel FA, Moschella SL
J Am Acad Dermatol 1984 Jul;11(1):1-19. doi: 10.1016/s0190-9622(84)70133-2. PMID: 6376553
Lerner CW, Tapper ML
Medicine (Baltimore) 1984 May;63(3):155-64. doi: 10.1097/00005792-198405000-00002. PMID: 6325849
Goldblum SE, Reed WP
Ann Intern Med 1980 Oct;93(4):597-613. doi: 10.7326/0003-4819-93-4-597. PMID: 7001976
Miglets AW, Viall JH, Kataria YP
Laryngoscope 1977 Dec;87(12):2038-48. doi: 10.1288/00005537-197712000-00009. PMID: 926968

Therapy

Ohrui T
Tohoku J Exp Med 2005 Sep;207(1):3-12. doi: 10.1620/tjem.207.3. PMID: 16082150
MMWR Recomm Rep 1997 Sep 5;46(RR-15):1-10. PMID: 9332982
Kerdel FA, Moschella SL
J Am Acad Dermatol 1984 Jul;11(1):1-19. doi: 10.1016/s0190-9622(84)70133-2. PMID: 6376553
Goldblum SE, Reed WP
Ann Intern Med 1980 Oct;93(4):597-613. doi: 10.7326/0003-4819-93-4-597. PMID: 7001976
Paterson PY, Semo R, Blumenschein G, Swelstad J
Clin Exp Immunol 1971 Nov;9(5):595-602. PMID: 4947556Free PMC Article

Prognosis

Harris TG, Burk RD, Xue X, Anastos K, Minkoff H, Massad LS, Young MA, Levine AM, Gange SJ, Watts DH, Palefsky JM, Strickler HD
AIDS 2007 Sep 12;21(14):1933-41. doi: 10.1097/QAD.0b013e3282c3a945. PMID: 17721101
Shankar MS, Aravindan AN, Sohal PM, Kohli HS, Sud K, Gupta KL, Sakhuja V, Jha V
Nephrol Dial Transplant 2005 Dec;20(12):2720-4. Epub 2005 Sep 27 doi: 10.1093/ndt/gfi141. PMID: 16188895
Soave R, Danner RL, Honig CL, Ma P, Hart CC, Nash T, Roberts RB
Ann Intern Med 1984 Apr;100(4):504-11. doi: 10.7326/0003-4819-100-4-504. PMID: 6703542
Kerdel FA, Moschella SL
J Am Acad Dermatol 1984 Jul;11(1):1-19. doi: 10.1016/s0190-9622(84)70133-2. PMID: 6376553
Eilber FR, Morton DL
Surg Forum 1969;20:116-7. PMID: 5383024

Clinical prediction guides

Shankar MS, Aravindan AN, Sohal PM, Kohli HS, Sud K, Gupta KL, Sakhuja V, Jha V
Nephrol Dial Transplant 2005 Dec;20(12):2720-4. Epub 2005 Sep 27 doi: 10.1093/ndt/gfi141. PMID: 16188895
Bennett BK, Hickie IB, Vollmer-Conna US, Quigley B, Brennan CM, Wakefield D, Douglas MP, Hansen GR, Tahmindjis AJ, Lloyd AR
Aust N Z J Psychiatry 1998 Apr;32(2):180-6. doi: 10.3109/00048679809062727. PMID: 9588296
Graham NM, Galai N, Nelson KE, Astemborski J, Bonds M, Rizzo RT, Sheeley L, Vlahov D
Arch Intern Med 1996 Apr 22;156(8):889-94. PMID: 8774208
Mukadi Y, Perriëns JH, St Louis ME, Brown C, Prignot J, Willame JC, Pouthier F, Kaboto M, Ryder RW, Portaels F
Lancet 1993 Jul 17;342(8864):143-6. doi: 10.1016/0140-6736(93)91346-n. PMID: 8101257
Pamphilon DH, Alnaqdy AA, Wallington TB
Immunol Today 1991 Apr;12(4):119-23. doi: 10.1016/0167-5699(91)90095-B. PMID: 2059312

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