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Recurrent aphthous stomatitis

MedGen UID:
445425
Concept ID:
C2937365
Disease or Syndrome
Synonym: Canker sore
SNOMED CT: Recurrent aphthous ulcer (398870000); Recurrent aphthous ulceration (398870000); Recurrent aphthous stomatitis (722781002); Recurrent oral aphthae (722781002); Recurrent ulcerative stomatitis (722781002)
 
HPO: HP:0011107
Monarch Initiative: MONDO:0005318

Definition

Recurrent episodes of ulceration of the oral mucosa, typically presenting as painful, sharply circumscribed fibrin-covered mucosal defects with a hyperemic border. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent aphthous stomatitis

Conditions with this feature

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.
Familial amyloid nephropathy with urticaria AND deafness
MedGen UID:
120634
Concept ID:
C0268390
Disease or Syndrome
Muckle-Wells syndrome (MWS) is characterized by episodic skin rash, arthralgias, and fever associated with late-onset sensorineural deafness and renal amyloidosis (Dode et al., 2002).
Lazy leukocyte syndrome
MedGen UID:
78795
Concept ID:
C0272174
Disease or Syndrome
Periodic fever, immunodeficiency, and thrombocytopenia syndrome (PFITS) is an autosomal recessive immunologic disorder with variable manifestations. Common features include early-onset recurrent respiratory infections, stomatitis, and cutaneous infections. Organisms usually include bacteria such as pneumococcus, Staphylococcus, and H. influenzae, but severe viral infections, including varicella, may also occur. Laboratory investigations may show neutropenia, neutrophilia, leukocytosis, or lymphopenia, although levels of immune cells may also be normal. Detailed studies often show impaired neutrophil chemotaxis associated with increased or abnormal F-actin levels, and impaired, normal, or even increased oxidative burst, depending on the stimulus. B- and T-cell abnormalities have also been observed. Some patients develop autoimmune manifestations, including chronic thrombocytopenia, anemia, and periodic fevers, associated with activation of the inflammasome. Early death may occur; however, hematopoietic stem cell transplantation may be curative (summary by Kuhns et al., 2016, Standing et al., 2017, and Pfajfer et al., 2018).
Pelger-Huet-like anomaly and episodic fever with abdominal pain
MedGen UID:
376692
Concept ID:
C1850054
Disease or Syndrome
Immunodeficiency-108 with autoinflammation (IMD108) is an autosomal recessive disorder characterized mainly by features of autoinflammation, often manifest as onset of recurrent episodes of abdominal pain associated with fever and elevated inflammatory markers around adolescence. Affected individuals also have recurrent infections, particularly of the skin and nails; poor wound healing; and mild bleeding tendencies. Peripheral blood examination shows hypolobulated neutrophils, suggesting a defect in myeloid differentiation and function. However, neutrophil primary and secondary granules are normal (summary by Goos et al., 2019).
Granulocytopenia with immunoglobulin abnormality
MedGen UID:
383874
Concept ID:
C1856263
Disease or Syndrome
Immunodeficiency-59 and hypoglycemia (IMD59) is an autosomal recessive primary immunologic disorder characterized by combined immunodeficiency and recurrent septic infections of the respiratory tract, skin, and mucous membranes, as well as disturbed glucose metabolism. Granulocytopenia and B-cell and dendritic cell deficiency are present (Haapaniemi et al., 2017).
Celiac disease, susceptibility to, 1
MedGen UID:
395227
Concept ID:
C1859310
Finding
Celiac disease is a systemic autoimmune disease that can be associated with gastrointestinal findings (diarrhea, malabsorption, abdominal pain and distension, bloating, vomiting, and weight loss) and/or highly variable non-gastrointestinal findings (dermatitis herpetiformis, chronic fatigue, joint pain/inflammation, iron deficiency anemia, migraines, depression, attention-deficit disorder, epilepsy, osteoporosis/osteopenia, infertility and/or recurrent fetal loss, vitamin deficiencies, short stature, failure to thrive, delayed puberty, dental enamel defects, and autoimmune disorders). Classic celiac disease, characterized by mild to severe gastrointestinal symptoms, is less common than non-classic celiac disease, characterized by absence of gastrointestinal symptoms.
Familial cold autoinflammatory syndrome 2
MedGen UID:
435869
Concept ID:
C2673198
Disease or Syndrome
Familial cold autoinflammatory syndrome-2 (FCAS2) is an autosomal dominant autoinflammatory disorder characterized by episodic and recurrent rash, urticaria, arthralgia, myalgia, and headache. In most patients, these episodes are accompanied by fever and serologic evidence of inflammation. Most, but not all, patients report exposure to cold as a trigger for the episodes. Additional features may include abdominal pain, thoracic pain, and sensorineural deafness. The age at onset is variable, ranging from the first year of life to middle age, and the severity and clinical manifestations are heterogeneous (summary by Shen et al., 2017). For a phenotypic description and a discussion of genetic heterogeneity of familial cold autoinflammatory syndrome, see FCAS1 (120100).
Combined immunodeficiency due to ORAI1 deficiency
MedGen UID:
440578
Concept ID:
C2748568
Disease or Syndrome
Immunodeficiency-9 (IMD9) is an autosomal recessive disorder characterized by early onset of recurrent infections due to defective T-cell activation. Affected individuals also have congenital myopathy resulting in muscle weakness as well as features of ectodermal dysplasia, including soft dental enamel (summary by McCarl et al., 2009).
RIN2 syndrome
MedGen UID:
416526
Concept ID:
C2751321
Disease or Syndrome
A very rare inherited connective tissue disorder with characteristics of macrocephaly, sparse scalp hair, soft redundant and hyperextensible skin, joint hypermobility, and scoliosis. Patients have progressive facial coarsening with downslanted palpebral fissures, upper eyelid fullness/infraorbital folds, thick/everted vermillion, gingival overgrowth and abnormal position of the teeth. Rare manifestations such as abnormal high-pitched voice, bronchiectasis, hypergonadotropic hypergonadism and brachydactyly have also been reported. Caused by homozygous mutation in the RIN2 gene on chromosome 20p11.
Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 3
MedGen UID:
462759
Concept ID:
C3151409
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Herpes simplex encephalitis, susceptibility to, 4
MedGen UID:
766783
Concept ID:
C3553869
Finding
A Mendelian susceptibility or predisposition to herpes simplex infection induced encephalitis in which the cause of the diseas is a mutation in the TICAM1 gene.
Combined immunodeficiency due to STK4 deficiency
MedGen UID:
766857
Concept ID:
C3553943
Disease or Syndrome
Immunodeficiency-110 (IMD110) is an autosomal recessive primary T-cell immunodeficiency syndrome characterized by progressive loss of naive T cells, recurrent bacterial, viral, and fungal infections, warts, and abscesses, and autoimmune manifestations. Patients are at risk for developing lymphoproliferative disorders or lymphoma, particularly associated with EBV. Some patients may show cardiac malformations, including atrial septal defect (Abdollahpour et al., 2012; Nehme et al., 2012).
Combined immunodeficiency due to MALT1 deficiency
MedGen UID:
815913
Concept ID:
C3809583
Disease or Syndrome
Combined immunodeficiency due to MALT1 deficiency is a rare, genetic form of primary immunodeficiency characterized by growth retardation, early recurrent pulmonary infections leading to bronchiectasis, inflammatory gastrointestinal disease, and other symptoms, such as rash, dermatitis, skin infections.
Severe combined immunodeficiency due to DNA-PKcs deficiency
MedGen UID:
863270
Concept ID:
C4014833
Disease or Syndrome
Severe combined immunodeficiency (SCID) due to DNA-PKcs deficiency is an extremely rare type of SCID (see this term) characterized by the classical signs of SCID (severe and recurrent infections, diarrhea, failure to thrive), absence of T and B lymphocytes, and cell sensitivity to ionizing radiation.
Tenorio syndrome
MedGen UID:
864147
Concept ID:
C4015710
Disease or Syndrome
Tenorio syndrome is characterized by overgrowth, macrocephaly, and intellectual disability (ID). Some patients may have mild hydrocephaly, hypoglycemia, and inflammatory diseases resembling Sjogren syndrome (270150) (summary by Tenorio et al., 2014).
Candidiasis, familial, 9
MedGen UID:
906897
Concept ID:
C4225324
Disease or Syndrome
Any chronic mucocutaneous candidiasis in which the cause of the disease is a mutation in the IL17RC gene.
Severe combined immunodeficiency due to CARMIL2 deficiency
MedGen UID:
1648422
Concept ID:
C4748304
Disease or Syndrome
Immunodeficiency-58 is an autosomal recessive primary immunologic disorder characterized by early-onset skin lesions, including eczematous dermatitis, infectious abscesses, and warts, recurrent respiratory infections or allergies, and chronic persistent infections with candida, Molluscum contagiosum, mycobacteria, EBV, bacteria, and viruses. Some patients may have gastrointestinal involvement, including inflammatory bowel disease, EBV+ smooth muscle tumors, and esophagitis. Immunologic analysis shows defective T-cell function with decreased Treg cells and deficient CD3/CD28 costimulation responses in both CD4+ and CD8+ T cells. B-cell function may also be impaired (summary by Wang et al., 2016 and Alazami et al., 2018).
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to complete RORgamma receptor deficiency
MedGen UID:
1799070
Concept ID:
C5567647
Disease or Syndrome
Immunodeficiency-42 is an autosomal recessive primary immunodeficiency characterized by increased susceptibility to mycobacterial and candidal infections beginning in infancy. Patients vaccinated with BCG are particularly at risk for developing disseminated mycobacterial infections (summary by Okada et al., 2015).
Immunodeficiency 98 with autoinflammation, X-linked
MedGen UID:
1805285
Concept ID:
C5676883
Disease or Syndrome
X-linked immunodeficiency-98 with autoinflammation (IMD98) is characterized by onset of recurrent infections associated with lymphoproliferation and autoinflammation in the first decade of life. Mostly males are affected; carrier females may have mild symptoms. Laboratory studies show evidence of immune dysregulation, including hypogammaglobulinemia with reduced memory B cells, skewed T-cell subsets, increased levels of proinflammatory cytokines, activated T cells and monocytes, and autoimmune cytopenias, including neutropenia (Aluri et al., 2021; Fejtkova et al., 2022).

Professional guidelines

PubMed

Milia E, Sotgiu MA, Spano G, Filigheddu E, Gallusi G, Campanella V
Eur J Paediatr Dent 2022 Mar;23(1):73-78. doi: 10.23804/ejpd.2022.23.01.14. PMID: 35274547
Gasmi Benahmed A, Noor S, Menzel A, Gasmi A
Arch Razi Inst 2021 Nov;76(5):1155-1163. Epub 2021 Nov 30 doi: 10.22092/ari.2021.356055.1767. PMID: 35355774Free PMC Article
Chiang CP, Yu-Fong Chang J, Wang YP, Wu YH, Wu YC, Sun A
J Formos Med Assoc 2019 Sep;118(9):1279-1289. Epub 2018 Nov 14 doi: 10.1016/j.jfma.2018.10.023. PMID: 30446298

Recent clinical studies

Etiology

Liu H, Tan L, Fu G, Chen L, Tan H
Medicina (Kaunas) 2022 Jun 7;58(6) doi: 10.3390/medicina58060771. PMID: 35744034Free PMC Article
Randall DA, Wilson Westmark NL, Neville BW
Am Fam Physician 2022 Apr 1;105(4):369-376. PMID: 35426641
Gutierrez Gossweiler A, Martinez-Mier EA
Monogr Oral Sci 2020;28:59-67. Epub 2019 Nov 7 doi: 10.1159/000455372. PMID: 31940621
Suter VGA, Sjölund S, Bornstein MM
Lasers Med Sci 2017 May;32(4):953-963. Epub 2017 Mar 27 doi: 10.1007/s10103-017-2184-z. PMID: 28345122
Akintoye SO, Greenberg MS
Dent Clin North Am 2014 Apr;58(2):281-97. Epub 2014 Jan 21 doi: 10.1016/j.cden.2013.12.002. PMID: 24655523Free PMC Article

Diagnosis

Conejero Del Mazo R, García Forcén L, Navarro Aguilar ME
Med Clin (Barc) 2023 Sep 29;161(6):251-259. Epub 2023 Jun 23 doi: 10.1016/j.medcli.2023.05.007. PMID: 37357066
Lau CB, Smith GP
Dermatol Ther 2022 Jun;35(6):e15500. Epub 2022 Apr 18 doi: 10.1111/dth.15500. PMID: 35395126
Gasmi Benahmed A, Noor S, Menzel A, Gasmi A
Arch Razi Inst 2021 Nov;76(5):1155-1163. Epub 2021 Nov 30 doi: 10.22092/ari.2021.356055.1767. PMID: 35355774Free PMC Article
Sánchez-Bernal J, Conejero C, Conejero R
Actas Dermosifiliogr (Engl Ed) 2020 Jul-Aug;111(6):471-480. Epub 2020 May 22 doi: 10.1016/j.ad.2019.09.004. PMID: 32451064
Akintoye SO, Greenberg MS
Dent Clin North Am 2014 Apr;58(2):281-97. Epub 2014 Jan 21 doi: 10.1016/j.cden.2013.12.002. PMID: 24655523Free PMC Article

Therapy

Liu H, Tan L, Fu G, Chen L, Tan H
Medicina (Kaunas) 2022 Jun 7;58(6) doi: 10.3390/medicina58060771. PMID: 35744034Free PMC Article
Lau CB, Smith GP
Dermatol Ther 2022 Jun;35(6):e15500. Epub 2022 Apr 18 doi: 10.1111/dth.15500. PMID: 35395126
Amorim Dos Santos J, Normando AGC, de Toledo IP, Melo G, De Luca Canto G, Santos-Silva AR, Guerra ENS
Clin Oral Investig 2020 Jan;24(1):37-45. Epub 2019 Nov 12 doi: 10.1007/s00784-019-03144-z. PMID: 31720851
Suter VGA, Sjölund S, Bornstein MM
Lasers Med Sci 2017 May;32(4):953-963. Epub 2017 Mar 27 doi: 10.1007/s10103-017-2184-z. PMID: 28345122
Ship JA, Chavez EM, Doerr PA, Henson BS, Sarmadi M
Quintessence Int 2000 Feb;31(2):95-112. PMID: 11203919

Prognosis

Yousefi H, Gholami M, Zoughi M, Rezaei N, Chuppani A, Nikfar S, Amoli MM
Cytokine 2022 May;153:155864. Epub 2022 Mar 18 doi: 10.1016/j.cyto.2022.155864. PMID: 35313259
Han Y, Wang L, Li Q, Chen H, Ma X
BMC Oral Health 2021 Dec 31;21(1):673. doi: 10.1186/s12903-021-01909-1. PMID: 34972503Free PMC Article
Gomes CC, Gomez RS, Zina LG, Amaral FR
Med Oral Patol Oral Cir Bucal 2016 Mar 1;21(2):e187-91. doi: 10.4317/medoral.20872. PMID: 26827061Free PMC Article
Landová H, Daněk Z, Gajdziok J, Vetchý D, Stembírek J
Ceska Slov Farm 2013 Feb;62(1):12-8. PMID: 23578262
Stoopler ET, Musbah T
CMAJ 2013 Mar 19;185(5):E240. Epub 2012 Sep 10 doi: 10.1503/cmaj.120706. PMID: 22966062Free PMC Article

Clinical prediction guides

Randall DA, Wilson Westmark NL, Neville BW
Am Fam Physician 2022 Apr 1;105(4):369-376. PMID: 35426641
Girardelli M, Valencic E, Moressa V, Margagliotta R, Tesser A, Pastore S, Spadola O, Athanasakis E, Severini GM, Taddio A, Tommasini A
Pediatr Rheumatol Online J 2021 May 10;19(1):70. doi: 10.1186/s12969-021-00552-y. PMID: 33971891Free PMC Article
Suter VGA, Sjölund S, Bornstein MM
Lasers Med Sci 2017 May;32(4):953-963. Epub 2017 Mar 27 doi: 10.1007/s10103-017-2184-z. PMID: 28345122
Gomes CC, Gomez RS, Zina LG, Amaral FR
Med Oral Patol Oral Cir Bucal 2016 Mar 1;21(2):e187-91. doi: 10.4317/medoral.20872. PMID: 26827061Free PMC Article
Ship JA, Chavez EM, Doerr PA, Henson BS, Sarmadi M
Quintessence Int 2000 Feb;31(2):95-112. PMID: 11203919

Recent systematic reviews

Surboyo MDC, Boedi RM, Hariyani N, Santosh ABR, Manuaba IBPP, Cecilia PH, Ambarawati IGAD, Parmadiati AE, Ernawati DS
Cytokine 2022 Sep;157:155946. Epub 2022 Jun 18 doi: 10.1016/j.cyto.2022.155946. PMID: 35728503
Al-Maweri SA, Alaizari N, Alharbi AA, Alotaibi SA, AlQuhal A, Almutairi BF, Alhuthaly S, Almutairi AM
J Dermatolog Treat 2022 May;33(3):1225-1230. Epub 2020 Sep 9 doi: 10.1080/09546634.2020.1819529. PMID: 32893718
Al-Maweri SA, Alaizari N, Alanazi RH, Ashraf S, Alanazi RH, Al-Sharani HM, Halboub E
Clin Oral Investig 2021 Dec;25(12):6561-6570. Epub 2021 Sep 20 doi: 10.1007/s00784-021-04180-4. PMID: 34542725
Suter VGA, Sjölund S, Bornstein MM
Lasers Med Sci 2017 May;32(4):953-963. Epub 2017 Mar 27 doi: 10.1007/s10103-017-2184-z. PMID: 28345122
Gomes CC, Gomez RS, Zina LG, Amaral FR
Med Oral Patol Oral Cir Bucal 2016 Mar 1;21(2):e187-91. doi: 10.4317/medoral.20872. PMID: 26827061Free PMC Article

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