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Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation(APLAID)

MedGen UID:
766875
Concept ID:
C3553961
Disease or Syndrome
Synonyms: AUTOINFLAMMATION, ANTIBODY DEFICIENCY, AND IMMUNE DYSREGULATION; Autoinflammation, antibody deficiency, and immune dysregulation syndrome; Autoinflammation, antibody deficiency, and immune dysregulation, plcg2-associated
SNOMED CT: Autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation (778004006); Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation (778004006); APLAID - autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation (778004006)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): PLCG2 (16q23.3)
 
Monarch Initiative: MONDO:0013944
OMIM®: 614878
Orphanet: ORPHA324530

Definition

Autoinflammation, antibody deficiency, and immune dysregulation (APLAID) is an autosomal dominant systemic disorder characterized by recurrent blistering skin lesions with a dense inflammatory infiltrate and variable involvement of other tissues, including joints, the eye, and the gastrointestinal tract. Affected individuals have a mild humoral immune deficiency associated with recurrent sinopulmonary infections, but no evidence of circulating autoantibodies (summary by Zhou et al., 2012). [from OMIM]

Clinical features

From HPO
Arthralgia
MedGen UID:
13917
Concept ID:
C0003862
Sign or Symptom
Joint pain.
Ulcerative colitis
MedGen UID:
3532
Concept ID:
C0009324
Disease or Syndrome
A chronic inflammatory bowel disease that includes characteristic ulcers, or open sores, in the colon. The main symptom of active disease is usually constant diarrhea mixed with blood, of gradual onset and intermittent periods of exacerbated symptoms contrasting with periods that are relatively symptom-free. In contrast to Crohn's disease this special form of colitis begins in the distal parts of the rectum, spreads continually upwards and affects only mucose and submucose tissue of the colon.
Enterocolitis
MedGen UID:
4966
Concept ID:
C0014356
Disease or Syndrome
An inflammation of the colon and small intestine. However, most conditions are either categorized as Enteritis (inflammation of the small intestine) or Colitis (inflammation of the large intestine).
Cellulitis
MedGen UID:
40174
Concept ID:
C0007642
Disease or Syndrome
A bacterial infection and inflammation of the skin und subcutaneous tissues.
Bronchiolitis
MedGen UID:
14235
Concept ID:
C0006271
Disease or Syndrome
Inflammation of the bronchioles.
Interstitial pneumonitis
MedGen UID:
61507
Concept ID:
C0206061
Disease or Syndrome
Inflammation of interstitial lung tissue, usually associated with infection.
Recurrent sinopulmonary infections
MedGen UID:
339549
Concept ID:
C1846546
Finding
An increased susceptibility to infections involving both the paranasal sinuses and the lungs, as manifested by a history of recurrent sinopulmonary infections.
Decreased circulating IgA concentration
MedGen UID:
57934
Concept ID:
C0162538
Disease or Syndrome
Decreased levels of immunoglobulin A (IgA).
Decreased circulating total IgM
MedGen UID:
116095
Concept ID:
C0239989
Finding
An abnormally decreased level of immunoglobulin M (IgM) in blood.
Immune dysregulation
MedGen UID:
335001
Concept ID:
C1844666
Finding
Altered immune function characterized by lymphoid proliferation, immune activation, and excessive autoreactivity often leading to autoimmune/inflammatory complications.
Decreased proportion of class-switched memory B cells
MedGen UID:
892709
Concept ID:
C4072925
Finding
A reduction in the normal proportion of class-switched memory B cells (CD19+/CD27+/IgM+/IgD+) relative to the total number of B cells. Marginal zone B cells undergo limited somatic hypermutation and produce high-affinity IgM and some IgG, whereas class-switched memory B cells synthetize IgG, IgM, and IgA.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.
Cataract
MedGen UID:
39462
Concept ID:
C0086543
Disease or Syndrome
A cataract is an opacity or clouding that develops in the crystalline lens of the eye or in its capsule.
Corneal erosion
MedGen UID:
97882
Concept ID:
C0392163
Disease or Syndrome
An erosion or abrasion of the cornea's outermost layer of epithelial cells.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
Follow this link to review classifications for Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation in Orphanet.

Recent clinical studies

Etiology

Almarzooqi F, Souid AK, Vijayan R, Al-Hammadi S
PLoS One 2021;16(1):e0245888. Epub 2021 Jan 22 doi: 10.1371/journal.pone.0245888. PMID: 33481921Free PMC Article
Magen E, Schlesinger M, David M, Ben-Zion I, Vardy D
Allergy Asthma Proc 2014 Mar-Apr;35(2):e27-33. doi: 10.2500/aap.2014.35.3734. PMID: 24717782

Diagnosis

Almarzooqi F, Souid AK, Vijayan R, Al-Hammadi S
PLoS One 2021;16(1):e0245888. Epub 2021 Jan 22 doi: 10.1371/journal.pone.0245888. PMID: 33481921Free PMC Article
Magen E, Schlesinger M, David M, Ben-Zion I, Vardy D
Allergy Asthma Proc 2014 Mar-Apr;35(2):e27-33. doi: 10.2500/aap.2014.35.3734. PMID: 24717782

Therapy

Magen E, Schlesinger M, David M, Ben-Zion I, Vardy D
Allergy Asthma Proc 2014 Mar-Apr;35(2):e27-33. doi: 10.2500/aap.2014.35.3734. PMID: 24717782

Prognosis

Almarzooqi F, Souid AK, Vijayan R, Al-Hammadi S
PLoS One 2021;16(1):e0245888. Epub 2021 Jan 22 doi: 10.1371/journal.pone.0245888. PMID: 33481921Free PMC Article

Clinical prediction guides

Almarzooqi F, Souid AK, Vijayan R, Al-Hammadi S
PLoS One 2021;16(1):e0245888. Epub 2021 Jan 22 doi: 10.1371/journal.pone.0245888. PMID: 33481921Free PMC Article

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