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Gingivitis

MedGen UID:
4895
Concept ID:
C0017574
Disease or Syndrome
Synonym: Gingivitides
SNOMED CT: Gingivitis (66383009)
 
HPO: HP:0000230
Monarch Initiative: MONDO:0002508

Definition

Inflammation of the gingiva. [from HPO]

Conditions with this feature

Chédiak-Higashi syndrome
MedGen UID:
3347
Concept ID:
C0007965
Disease or Syndrome
Chediak-Higashi syndrome (CHS) is characterized by partial oculocutaneous albinism, immunodeficiency, and a mild bleeding tendency. Approximately 85% of affected individuals develop the accelerated phase, or hemophagocytic lymphohistiocytosis, a life-threatening, hyperinflammatory condition. All affected individuals including adolescents and adults with atypical CHS and children with classic CHS who have successfully undergone allogenic hematopoietic stem cell transplantation (HSCT) develop neurologic findings during early adulthood.
Alstrom syndrome
MedGen UID:
78675
Concept ID:
C0268425
Disease or Syndrome
Alström syndrome is characterized by cone-rod dystrophy, obesity, progressive bilateral sensorineural hearing impairment, acute infantile-onset cardiomyopathy and/or adolescent- or adult-onset restrictive cardiomyopathy, insulin resistance / type 2 diabetes mellitus (T2DM), nonalcoholic fatty liver disease (NAFLD), and chronic progressive kidney disease. Cone-rod dystrophy presents as progressive visual impairment, photophobia, and nystagmus usually starting between birth and age 15 months. Many individuals lose all perception of light by the end of the second decade, but a minority retain the ability to read large print into the third decade. Children usually have normal birth weight but develop truncal obesity during their first year. Sensorineural hearing loss presents in the first decade in as many as 70% of individuals and may progress to the severe or moderately severe range (40-70 db) by the end of the first to second decade. Insulin resistance is typically accompanied by the skin changes of acanthosis nigricans, and proceeds to T2DM in the majority by the third decade. Nearly all demonstrate hypertriglyceridemia. Other findings can include endocrine abnormalities (hypothyroidism, hypogonadotropic hypogonadism in males, and hyperandrogenism in females), urologic dysfunction / detrusor instability, progressive decrease in renal function, and hepatic disease (ranging from elevated transaminases to steatohepatitis/NAFLD). Approximately 20% of affected individuals have delay in early developmental milestones, most commonly in gross and fine motor skills. About 30% have a learning disability. Cognitive impairment (IQ <70) is very rare. Wide clinical variability is observed among affected individuals, even within the same family.
Hyper-IgM syndrome type 1
MedGen UID:
96019
Concept ID:
C0398689
Disease or Syndrome
X-linked hyper IgM syndrome (HIGM1), a disorder of abnormal T- and B-cell function, is characterized by low serum concentrations of IgG, IgA, and IgE with normal or elevated serum concentrations of IgM. Mitogen proliferation may be normal, but NK- and T-cell cytotoxicity can be impaired. Antigen-specific responses are usually decreased or absent. Total numbers of B cells are normal but there is a marked reduction of class-switched memory B cells. Defective oxidative burst of both neutrophils and macrophages has been reported. The range of clinical findings varies, even within the same family. More than 50% of males with HIGM1 develop symptoms by age one year, and more than 90% are symptomatic by age four years. HIGM1 usually presents in infancy with recurrent upper- and lower-respiratory tract bacterial infections, opportunistic infections including Pneumocystis jirovecii pneumonia, and recurrent or protracted diarrhea that can be infectious or noninfectious and is associated with failure to thrive. Neutropenia is common; thrombocytopenia and anemia are less commonly seen. Autoimmune and/or inflammatory disorders (such as sclerosing cholangitis) as well as increased risk for neoplasms have been reported as medical complications of this disorder. Significant neurologic complications, often the result of a CNS infection, are seen in 5%-15% of affected males. Liver disease, a serious complication of HIGM1 once observed in more than 80% of affected males by age 20 years, may be decreasing with adequate screening and treatment of Cryptosporidium infection.
Leukocyte adhesion deficiency 1
MedGen UID:
98310
Concept ID:
C0398738
Disease or Syndrome
Leukocyte adhesion deficiency (LAD) is an autosomal recessive disorder of neutrophil function resulting from a deficiency of the beta-2 integrin subunit of the leukocyte cell adhesion molecule. The leukocyte cell adhesion molecule is present on the surface of peripheral blood mononuclear leukocytes and granulocytes and mediates cell-cell and cell-extracellular matrix adhesion. LAD is characterized by recurrent bacterial infections; impaired pus formation and wound healing; abnormalities of a wide variety of adhesion-dependent functions of granulocytes, monocytes, and lymphocytes; and a lack of beta-2/alpha-L, beta-2/alpha-M, and beta-2/alpha-X expression. Genetic Heterogeneity of Leukocyte Adhesion Deficiency Also see LAD2 (266265), caused by mutation in the SLC35C1 gene (605881), and LAD3 (612840), caused by mutation in the FERMT3 gene (607901).
Kindler syndrome
MedGen UID:
96060
Concept ID:
C0406557
Disease or Syndrome
Kindler syndrome (KS), a rare subtype of inherited epidermolysis bullosa, is characterized by skin fragility and acral blister formation beginning at birth, diffuse cutaneous atrophy, photosensitivity (most prominent during childhood and usually decreasing after adolescence), poikiloderma, diffuse palmoplantar hyperkeratosis, and pseudosyndactyly. Mucosal manifestations are also common and include hemorrhagic mucositis and gingivitis, periodontal disease, premature loss of teeth, and labial leukokeratosis. Other mucosal findings can include ectropion, urethral stenosis, and severe phimosis. Severe long-term complications of KS include periodontitis, mucosal strictures, and aggressive squamous cell carcinomas. Manifestations can range from mild to severe.
Normophosphatemic familial tumoral calcinosis
MedGen UID:
355311
Concept ID:
C1864861
Disease or Syndrome
Plasminogen deficiency, type I
MedGen UID:
369859
Concept ID:
C1968804
Disease or Syndrome
Congenital plasminogen deficiency is a rare autosomal recessive disorder characterized clinically by chronic mucosal pseudomembranous lesions consisting of subepithelial fibrin deposition and inflammation. The most common clinical manifestation is ligneous ('wood-like') conjunctivitis, a redness and subsequent formation of pseudomembranes mostly on the palpebral surfaces of the eye that progress to white, yellow-white, or red thick masses with a wood-like consistency that replace the normal mucosa. The lesions may be triggered by local injury and/or infection and often recur after local excision. Pseudomembranous lesions of other mucous membranes often occur in the mouth, nasopharynx, trachea, and female genital tract. Some affected children also have congenital occlusive hydrocephalus. A slightly increased female:male ratio has been observed (1.4:1 to 2:1) (Schuster and Seregard, 2003; Tefs et al., 2006). Type I plasminogen deficiency is characterized by decreased serum plasminogen activity, decreased plasminogen antigen levels, and clinical symptoms, whereas type II plasminogen deficiency, also known as 'dysplasminogenemia,' is characterized by decreased plasminogen activity with normal or slightly reduced antigen levels. Patients with type II deficiency are usually asymptomatic. Ligneous conjunctivitis and pseudomembranous formation has only been associated with type I plasminogen deficiency. Presumably, normal amounts of plasminogen antigen with decreased activity, as seen in type II, is sufficient for normal wound healing (Schuster and Seregard, 2003).
Congenital ichthyosis-intellectual disability-spastic quadriplegia syndrome
MedGen UID:
482486
Concept ID:
C3280856
Disease or Syndrome
ISQMR is a severe autosomal recessive disorder characterized by ichthyosis apparent from birth, profound psychomotor retardation with essentially no development, spastic quadriplegia, and seizures (summary by Aldahmesh et al., 2011).
Chronic familial neutropenia
MedGen UID:
777137
Concept ID:
C3665676
Disease or Syndrome
Pachyonychia congenita 3
MedGen UID:
811523
Concept ID:
C3714948
Disease or Syndrome
Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar keratoderma and blistering, oral leukokeratosis, pilosebaceous cysts (including steatocystoma and vellus hair cysts), palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities.
Osteopetrosis, autosomal dominant 3
MedGen UID:
1648454
Concept ID:
C4748197
Disease or Syndrome
Autosomal dominant osteopetrosis-3 (OPTA3) is characterized by phenotypic variability. Some patients have typical features of osteopetrosis, including fractures after minor trauma, early tooth loss, anemia, hepatosplenomegaly, and a generalized increase in bone mineral density, whereas other patients exhibit localized osteosclerosis and generalized osteopenia. OPTA3 represents a relatively malignant form of osteopetrosis in some patients who develop significant pancytopenia and hepatosplenomegaly (Bo et al., 2016). For a discussion of genetic heterogeneity of autosomal dominant osteopetrosis, see OPTA1 (607634).
Immunodeficiency 65, susceptibility to viral infections
MedGen UID:
1684865
Concept ID:
C5231441
Disease or Syndrome
Immunodeficiency-65 (IMD65) is an autosomal recessive immunologic disorder characterized by onset of recurrent and severe viral infections from early infancy. Affected individuals have impaired ability to fight viral infections, resulting in clinically significant disease, including pneumonia, bronchiectasis, and septic shock. Laboratory studies may show lymphopenia or hypogammaglobulinemia, particularly during infection; more detailed studies show an impaired cellular type I interferon response. Treatment with intravenous immunoglobulin (IVIg) is beneficial. Important features of this disorder include the rapid development of septic shock, as well as poor outcomes after vaccination with live attenuated vaccines; such vaccines should never be administered to patients with known impaired interferon responses (summary by Hernandez et al., 2018 and Bravo Garcia-Morato et al., 2019).
Neutropenia, severe congenital, 11, autosomal dominant
MedGen UID:
1846394
Concept ID:
C5882742
Disease or Syndrome
Autosomal dominant severe congenital neutropenia-11 (SCN11) is characterized by the onset of recurrent infections, mainly bacterial, in early childhood. Laboratory studies show severe neutropenia due to maturation arrest and impaired development of myeloid cells. Other leukocyte subsets, including B cells and NK cells, may also be subtly affected. Patients should be followed for possible renal dysfunction (Van Nieuwenhove et al., 2020). For discussion of genetic heterogeneity of severe congenital neutropenia, see SCN1 (202700).

Professional guidelines

PubMed

Liu Y, Li CX, Nie J, Mi CB, Li YM
Chin J Dent Res 2023 Mar 29;26(1):11-18. doi: 10.3290/j.cjdr.b3978667. PMID: 36988062
Kumar S
Dent Clin North Am 2019 Jan;63(1):69-81. Epub 2018 Oct 29 doi: 10.1016/j.cden.2018.08.005. PMID: 30447793
Chapple IL, Van der Weijden F, Doerfer C, Herrera D, Shapira L, Polak D, Madianos P, Louropoulou A, Machtei E, Donos N, Greenwell H, Van Winkelhoff AJ, Eren Kuru B, Arweiler N, Teughels W, Aimetti M, Molina A, Montero E, Graziani F
J Clin Periodontol 2015 Apr;42 Suppl 16:S71-6. doi: 10.1111/jcpe.12366. PMID: 25639826

Recent clinical studies

Etiology

Revilla-León M, Gómez-Polo M, Barmak AB, Inam W, Kan JYK, Kois JC, Akal O
J Prosthet Dent 2023 Dec;130(6):816-824. Epub 2022 Mar 14 doi: 10.1016/j.prosdent.2022.01.026. PMID: 35300850
Zini A, Mazor S, Timm H, Barker ML, Grender JM, Gerlach RW, Biesbrock AR
Can J Dent Hyg 2021 Jun 1;55(2):85-94. PMID: 34221032Free PMC Article
Kumar S
Dent Clin North Am 2019 Jan;63(1):69-81. Epub 2018 Oct 29 doi: 10.1016/j.cden.2018.08.005. PMID: 30447793
Chapple ILC, Mealey BL, Van Dyke TE, Bartold PM, Dommisch H, Eickholz P, Geisinger ML, Genco RJ, Glogauer M, Goldstein M, Griffin TJ, Holmstrup P, Johnson GK, Kapila Y, Lang NP, Meyle J, Murakami S, Plemons J, Romito GA, Shapira L, Tatakis DN, Teughels W, Trombelli L, Walter C, Wimmer G, Xenoudi P, Yoshie H
J Periodontol 2018 Jun;89 Suppl 1:S74-S84. doi: 10.1002/JPER.17-0719. PMID: 29926944
Dufty J, Gkranias N, Donos N
Oral Health Prev Dent 2017;15(4):321-327. doi: 10.3290/j.ohpd.a38766. PMID: 28761942

Diagnosis

Younis RH, Georgaki M, Nikitakis NG
Oral Maxillofac Surg Clin North Am 2023 May;35(2):261-270. Epub 2023 Feb 15 doi: 10.1016/j.coms.2022.10.003. PMID: 36805902
Revilla-León M, Gómez-Polo M, Barmak AB, Inam W, Kan JYK, Kois JC, Akal O
J Prosthet Dent 2023 Dec;130(6):816-824. Epub 2022 Mar 14 doi: 10.1016/j.prosdent.2022.01.026. PMID: 35300850
Kumar S
Dent Clin North Am 2019 Jan;63(1):69-81. Epub 2018 Oct 29 doi: 10.1016/j.cden.2018.08.005. PMID: 30447793
Chapple ILC, Mealey BL, Van Dyke TE, Bartold PM, Dommisch H, Eickholz P, Geisinger ML, Genco RJ, Glogauer M, Goldstein M, Griffin TJ, Holmstrup P, Johnson GK, Kapila Y, Lang NP, Meyle J, Murakami S, Plemons J, Romito GA, Shapira L, Tatakis DN, Teughels W, Trombelli L, Walter C, Wimmer G, Xenoudi P, Yoshie H
J Periodontol 2018 Jun;89 Suppl 1:S74-S84. doi: 10.1002/JPER.17-0719. PMID: 29926944
Trombelli L, Farina R, Silva CO, Tatakis DN
J Clin Periodontol 2018 Jun;45 Suppl 20:S44-S67. doi: 10.1111/jcpe.12939. PMID: 29926492

Therapy

Worthington HV, MacDonald L, Poklepovic Pericic T, Sambunjak D, Johnson TM, Imai P, Clarkson JE
Cochrane Database Syst Rev 2019 Apr 10;4(4):CD012018. doi: 10.1002/14651858.CD012018.pub2. PMID: 30968949Free PMC Article
Iheozor-Ejiofor Z, Middleton P, Esposito M, Glenny AM
Cochrane Database Syst Rev 2017 Jun 12;6(6):CD005297. doi: 10.1002/14651858.CD005297.pub3. PMID: 28605006Free PMC Article
James P, Worthington HV, Parnell C, Harding M, Lamont T, Cheung A, Whelton H, Riley P
Cochrane Database Syst Rev 2017 Mar 31;3(3):CD008676. doi: 10.1002/14651858.CD008676.pub2. PMID: 28362061Free PMC Article
Yaacob M, Worthington HV, Deacon SA, Deery C, Walmsley AD, Robinson PG, Glenny AM
Cochrane Database Syst Rev 2014 Jun 17;2014(6):CD002281. doi: 10.1002/14651858.CD002281.pub3. PMID: 24934383Free PMC Article
Adams D, Addy M
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Prognosis

Pillai RS, Iyer K, Spin-Neto R, Kothari SF, Nielsen JF, Kothari M
Cerebrovasc Dis Extra 2018;8(1):1-15. Epub 2018 Jan 9 doi: 10.1159/000484989. PMID: 29402871Free PMC Article
Iheozor-Ejiofor Z, Middleton P, Esposito M, Glenny AM
Cochrane Database Syst Rev 2017 Jun 12;6(6):CD005297. doi: 10.1002/14651858.CD005297.pub3. PMID: 28605006Free PMC Article
Trombelli L, Farina R
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J Pediatr Hematol Oncol 2000 Nov-Dec;22(6):581-7. doi: 10.1097/00043426-200011000-00027. PMID: 11132234
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Clinical prediction guides

Pawlaczyk-Kamieńska T, Torlińska-Walkowiak N, Borysewicz-Lewicka M
Adv Clin Exp Med 2018 Oct;27(10):1397-1401. doi: 10.17219/acem/70417. PMID: 30058781
Trombelli L, Farina R, Silva CO, Tatakis DN
J Periodontol 2018 Jun;89 Suppl 1:S46-S73. doi: 10.1002/JPER.17-0576. PMID: 29926936
Trombelli L, Farina R, Silva CO, Tatakis DN
J Clin Periodontol 2018 Jun;45 Suppl 20:S44-S67. doi: 10.1111/jcpe.12939. PMID: 29926492
Kinane DF
Ann R Australas Coll Dent Surg 2000 Oct;15:34-41. PMID: 11709970
Newbrun E
J Periodontol 1996 Jun;67(6):555-61. doi: 10.1902/jop.1996.67.6.555. PMID: 8794964

Recent systematic reviews

Revilla-León M, Gómez-Polo M, Barmak AB, Inam W, Kan JYK, Kois JC, Akal O
J Prosthet Dent 2023 Dec;130(6):816-824. Epub 2022 Mar 14 doi: 10.1016/j.prosdent.2022.01.026. PMID: 35300850
Chen P, Hong F, Yu X
J Dent 2022 Oct;125:104253. Epub 2022 Aug 20 doi: 10.1016/j.jdent.2022.104253. PMID: 35998741
Van der Weijden FA, Van der Sluijs E, Ciancio SG, Slot DE
Dent Clin North Am 2015 Oct;59(4):799-829. doi: 10.1016/j.cden.2015.06.002. PMID: 26427569
Chapple IL, Van der Weijden F, Doerfer C, Herrera D, Shapira L, Polak D, Madianos P, Louropoulou A, Machtei E, Donos N, Greenwell H, Van Winkelhoff AJ, Eren Kuru B, Arweiler N, Teughels W, Aimetti M, Molina A, Montero E, Graziani F
J Clin Periodontol 2015 Apr;42 Suppl 16:S71-6. doi: 10.1111/jcpe.12366. PMID: 25639826
Yaacob M, Worthington HV, Deacon SA, Deery C, Walmsley AD, Robinson PG, Glenny AM
Cochrane Database Syst Rev 2014 Jun 17;2014(6):CD002281. doi: 10.1002/14651858.CD002281.pub3. PMID: 24934383Free PMC Article

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