Hairy leukoplakia

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Hairy leukoplakia
Other namesOral hairy leukoplakia, [1] : 385  OHL, or HIV-associated hairy leukoplakia [2]
Specialty Gastroenterology, dentistry   OOjs UI icon edit-ltr-progressive.svg

Hairy leukoplakia is a white patch on the side of the tongue with a corrugated or hairy appearance. It is caused by Epstein-Barr virus (EBV) and occurs usually in persons who are immunocompromised, especially those with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS). The white lesion, which cannot be scraped off, is benign and does not require any treatment, although its appearance may have diagnostic and prognostic implications for the underlying condition.

Contents

Depending upon what definition of leukoplakia is used, hairy leukoplakia is sometimes considered a subtype of leukoplakia, or a distinct diagnosis. [3]

Signs and symptoms

There are no symptoms associated with the lesion itself, [4] although many and varied symptoms and signs may be associated with the underlying cause of immunosuppression. The lesion is a white patch, which almost exclusively occurs on the lateral surfaces of the tongue, although rarely it may occur on the buccal mucosa, soft palate, pharynx or esophagus. [5] The lesion may grow to involve the dorsal surface of the tongue. The texture is vertically corrugated ("hairy") or thickly furrowed and shaggy in appearance. [5]

Causes

The white appearance is created by hyperkeratosis (overproduction of keratin) and epithelial hyperplasia. [5] The causative agent implicated is Epstein-Barr virus, the same virus that causes infectious mononucleosis (glandular fever). After the primary EBV infection has been overcome, the virus will persist for the rest of the host's life and "hides" from the immune system by latent infection of B lymphocytes. [6] The virus also causes lytic infection in the oropharynx, but is kept in check by a normal, functioning immune system. Uncontrolled lytic infection is manifested as oral hairy leukoplakia in immunocompromised hosts. OHL usually arises where the immunocompromise is secondary to HIV/AIDS. [5] Rarely are other causes of immunocompromise associated with OHL, but it has been reported in people who have received transplants and are taking immunosuppressive medication. OHL may also accompany chronic graft versus host disease. [7] Even more rare are reports of OHL in persons with competent immune systems. [5]

Diagnosis

The white lesion cannot be wiped away, [7] unlike some other common oral white lesions, e.g. pseudomembranous candidiasis, and this may aid in the diagnosis. Diagnosis of OHL is mainly clinical, but can be supported by proof of EBV in the lesion (achieved by in situ hybridization, polymerase chain reaction, immunohistochemistry, Southern blotting, or electron microscopy) and HIV serotesting. [7] When clinical appearance alone is used to diagnose OHL, there is a false positive rate of 17% compared to more objective methods. [8] The appearance of OHL in a person who is known to be infected with HIV does not usually require further diagnostic tests as the association is well known. OHL in persons with no known cause of immunocompromise usually triggers investigations to look for an underlying cause. If tissue biopsy is carried out, the histopathologic appearance is of hyperplastic and parakeratinized epithelium, with "balloon cells" (lightly staining cells) in the upper stratum spinosum and "nuclear beading" in the superficial layers (scattered cells with peripheral margination of chromatin and clear nuclei, created by displacement of chromatin to the peripheral nucleus by EBV replication). Candida usually is seen growing in the parakeratin layer, but there are no normal inflammatory reactions to this in the tissues. [5] There is no dysplasia (OHL is not a premalignant lesion). [5]

Classification

In a classification of the oral lesions in HIV disease, [9] OHL is grouped as "lesions strongly associated with HIV infection" (group I). [8] It could also be classed as an opportunistic, viral disease. Hairy leukoplakia occurs on the tongue and has a similar name to hairy tongue, but these are separate conditions with different causes.[ citation needed ]

Treatment

Treatment is not necessary since the lesion is benign, however, the person may have aesthetic concerns about the appearance. The condition often resolves rapidly with high dose acyclovir or desiclovir but recurs once this therapy is stopped, or as the underlying immunocompromise worsens. [8] [5] Topical use of podophyllum resin or retinoids has also been reported to produce temporary remission. [5]

Antiretroviral drugs such as zidovudine may be effective in producing a significant regression of OHL.[ clarification needed ] [5] Recurrence of the lesion may also signify that highly active antiretroviral therapy (HAART) is becoming ineffective. [4]

Prognosis

The oral lesion itself is benign and self-limiting, [7] however this may not necessarily be the case for the underlying cause of immunocompromise. For instance, OHL with HIV/AIDS is a predictor of bad prognosis, [7] (i.e. severe immunosuppression and advanced disease). [5]

Epidemiology

Hairy leukoplakia is one of the most common oral manifestations of HIV/AIDS, along with oral candidiasis. [7] It is the most common HIV/AIDS related condition caused by EBV, although EBV associated lymphomas may also occur. [5] OHL mainly occurs in adult males, less commonly in adult females and rarely in children. [8] The incidence rises as the CD4 count falls, [8] and the appearance of OHL may signify progression of HIV to AIDS. [10] A study from 2001 reported a significant decrease in the incidence of some oral manifestations of AIDS (including OHL and necrotizing ulcerative periodontitis), which was attributed to the use of HAART, whilst the incidence of other HIV-associated oral lesions did not alter significantly. [4]

History

Oral hairy leukoplakia was discovered by Deborah Greenspan and John S. Greenspan, and in 1985 they identified a connection between it and Epstein-Barr virus (EBV). [11] Oral hairy leukoplakia was first described by Greenspan et al. in 1984, [12] a few years after the start of the AIDS epidemic. A link with OHL was not initially reported as scientific understanding of HIV/AIDS was just beginning to develop at that time. It was subsequently thought to occur only in HIV-infected, homosexual males, however, this is now known to not always be the case. [8]

Research directions

It has been suggested that OHL be renamed according to its causative factor as “EBV leucoplakia”. [8]

Related Research Articles

<span class="mw-page-title-main">Epstein–Barr virus</span> Virus of the herpes family

The Epstein–Barr virus (EBV), formally called Human gammaherpesvirus 4, is one of the nine known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. EBV is a double-stranded DNA virus.

<span class="mw-page-title-main">Mouth ulcer</span> Sore on the mucous membrane of the oral cavity

A mouth ulcer (aphtha) is an ulcer that occurs on the mucous membrane of the oral cavity. Mouth ulcers are very common, occurring in association with many diseases and by many different mechanisms, but usually there is no serious underlying cause. Rarely, a mouth ulcer that does not heal may be a sign of oral cancer. These ulcers may form individually or multiple ulcers may appear at once. Once formed, an ulcer may be maintained by inflammation and/or secondary infection.

<span class="mw-page-title-main">Aciclovir</span> Antiviral medication used against herpes, chickenpox and shingles

Aciclovir (ACV), also known as acyclovir, is an antiviral medication. It is primarily used for the treatment of herpes simplex virus infections, chickenpox, and shingles. Other uses include prevention of cytomegalovirus infections following transplant and severe complications of Epstein–Barr virus infection. It can be taken by mouth, applied as a cream, or injected.

<span class="mw-page-title-main">Oral candidiasis</span> Fungal infection

Oral candidiasis, also known as oral thrush among other names, is candidiasis that occurs in the mouth. That is, oral candidiasis is a mycosis of Candida species on the mucous membranes of the mouth.

<span class="mw-page-title-main">Leukoplakia</span> Medical condition

Oral leukoplakia is a potentially malignant disorder affecting the oral mucosa. It is defined as "essentially an oral mucosal white lesion that cannot be considered as any other definable lesion." Oral leukoplakia is a white patch or plaque that develops in the oral cavity and is strongly associated with smoking. Leukoplakia is a firmly attached white patch on a mucous membrane which is associated with increased risk of cancer. The edges of the lesion are typically abrupt and the lesion changes with time. Advanced forms may develop red patches. There are generally no other symptoms. It usually occurs within the mouth, although sometimes mucosa in other parts of the gastrointestinal tract, urinary tract, or genitals may be affected.

This is a list of AIDS-related topics, many of which were originally taken from the public domain U.S. Department of Health Glossary of HIV/AIDS-Related Terms, 4th Edition.

Black hairy tongue syndrome (BHT) is a condition of the tongue in which the small bumps on the tongue elongate with black or brown discoloration, giving a black and hairy appearance. The appearance may be alarming, but it is a harmless condition. Predisposing factors include smoking, xerostomia, soft diet, poor oral hygiene and certain medications. Management is facilitated by improving oral hygiene, especially scraping or brushing the tongue.

The oral mucosa is the mucous membrane lining the inside of the mouth. It comprises stratified squamous epithelium, termed "oral epithelium", and an underlying connective tissue termed lamina propria. The oral cavity has sometimes been described as a mirror that reflects the health of the individual. Changes indicative of disease are seen as alterations in the oral mucosa lining the mouth, which can reveal systemic conditions, such as diabetes or vitamin deficiency, or the local effects of chronic tobacco or alcohol use. The oral mucosa tends to heal faster and with less scar formation compared to the skin. The underlying mechanism remains unknown, but research suggests that extracellular vesicles might be involved.

Immune reconstitution inflammatory syndrome (IRIS) is a condition seen in some cases of HIV/AIDS or immunosuppression, in which the immune system begins to recover, but then responds to a previously acquired opportunistic infection with an overwhelming inflammatory response that paradoxically makes the symptoms of infection worse.

<span class="mw-page-title-main">Primary effusion lymphoma</span> Medical condition

Primary effusion lymphoma (PEL) is classified as a diffuse large B cell lymphoma. It is a rare malignancy of plasmablastic cells that occurs in individuals that are infected with the Kaposi's sarcoma-associated herpesvirus. Plasmablasts are immature plasma cells, i.e. lymphocytes of the B-cell type that have differentiated into plasmablasts but because of their malignant nature do not differentiate into mature plasma cells but rather proliferate excessively and thereby cause life-threatening disease. In PEL, the proliferating plasmablastoid cells commonly accumulate within body cavities to produce effusions, primarily in the pleural, pericardial, or peritoneal cavities, without forming a contiguous tumor mass. In rare cases of these cavitary forms of PEL, the effusions develop in joints, the epidural space surrounding the brain and spinal cord, and underneath the capsule which forms around breast implants. Less frequently, individuals present with extracavitary primary effusion lymphomas, i.e., solid tumor masses not accompanied by effusions. The extracavitary tumors may develop in lymph nodes, bone, bone marrow, the gastrointestinal tract, skin, spleen, liver, lungs, central nervous system, testes, paranasal sinuses, muscle, and, rarely, inside the vasculature and sinuses of lymph nodes. As their disease progresses, however, individuals with the classical effusion-form of PEL may develop extracavitary tumors and individuals with extracavitary PEL may develop cavitary effusions.

<span class="mw-page-title-main">Tongue disease</span> Medical condition

Tongue diseases can be congenital or acquired, and are multiple in number. Considered according to a surgical sieve, some example conditions which can involve the tongue are discussed below. Glossitis is a general term for tongue inflammation, which can have various etiologies, e.g. infection.

There are several forms of Epstein–Barr virus (EBV) infection. These include asymptomatic infections, the primary infection, infectious mononucleosis, and the progression of asymptomatic or primary infections to: 1) any one of various Epstein–Barr virus-associated lymphoproliferative diseases such as chronic active EBV infection, EBV+ hemophagocytic lymphohistiocytosis, Burkitt's lymphoma, and Epstein–Barr virus positive diffuse large B-cell lymphoma, not otherwise specified); 2) non-lymphoid cancers such as Epstein–Barr virus associated gastric cancer, soft tissue sarcomas, leiomyosarcoma, and nasopharyngeal cancers; and 3) Epstein–Barr virus-associated non-lymphoproliferative diseases such as some cases of the immune disorders of multiple sclerosis and systemic lupus erythematosis and the childhood disorders of Alice in Wonderland Syndrome and acute cerebellar ataxia.

<span class="mw-page-title-main">Plasmablastic lymphoma</span> Type of large B-cell lymphoma

Plasmablastic lymphoma (PBL) is a type of large B-cell lymphoma recognized by the World Health Organization (WHO) in 2017 as belonging to a subgroup of lymphomas termed lymphoid neoplasms with plasmablastic differentiation. The other lymphoid neoplasms within this subgroup are: plasmablastic plasma cell lymphoma ; primary effusion lymphoma that is Kaposi's sarcoma-associated herpesvirus positive or Kaposi's sarcoma-associated Herpesvirus negative; anaplastic lymphoma kinase-positive large B-cell lymphoma; and human herpesvirus 8-positive diffuse large B-cell lymphoma, not otherwise specified. All of these lymphomas are malignancies of plasmablasts, i.e. B-cells that have differentiated into plasmablasts but because of their malignant nature: fail to differentiate further into mature plasma cells; proliferate excessively; and accumulate in and injure various tissues and organs.

Human immunodeficiency virus salivary gland disease, is swelling of the salivary glands and/or xerostomia in individuals infected with human immunodeficiency virus.

Deborah Greenspan is a British-American scholar in dentistry, having been Professor and the Leland and Gladys Barber Distinguished Professor at UCSF Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco.

Oral manifestations of systematic disease are signs and symptoms of disease occurring elsewhere in the body detected in the oral cavity and oral secretions. High blood sugar can be detected by sampling saliva. Saliva sampling may be a non-invasive way to detect changes in the gut microbiome and changes in systemic disease. Another example is tertiary syphilis, where changes to teeth can occur. Syphilis infection can be associated with longitudinal furrows of the tongue.

Epstein–Barr virus–associated lymphoproliferative diseases are a group of disorders in which one or more types of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells, are infected with the Epstein–Barr virus (EBV). This causes the infected cells to divide excessively, and is associated with the development of various non-cancerous, pre-cancerous, and cancerous lymphoproliferative disorders (LPDs). These LPDs include the well-known disorder occurring during the initial infection with the EBV, infectious mononucleosis, and the large number of subsequent disorders that may occur thereafter. The virus is usually involved in the development and/or progression of these LPDs although in some cases it may be an "innocent" bystander, i.e. present in, but not contributing to, the disease.

John S. Greenspan was an academic dentist/scientist and university administrator. His degrees and diplomas include BSc, BDS, Ph.D., FRCPath, FDSRCS (Eng). He was the Director-Emeritus of the AIDS Research Institute at the University of California, San Francisco (UCSF). He was also the founding Director of the UCSF AIDS Specimen Bank (1982-2017) and of the UCSF Oral AIDS Center (1986–2005).

Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) is a subtype of the Diffuse large B-cell lymphomas and a rare form of the Epstein–Barr virus-associated lymphoproliferative diseases, i.e. conditions in which lymphocytes infected with the Epstein-Barr virus (EBV) proliferate excessively in one or more tissues. EBV infects ~95% of the world's population to cause no symptoms, minor non-specific symptoms, or infectious mononucleosis. The virus then enters a latency phase in which the infected individual becomes a lifetime asymptomatic carrier of the virus. Some weeks, months, years, or decades thereafter, a very small fraction of these carriers, particularly those with an immunodeficiency, develop any one of various EBV-associated benign or malignant diseases.

<span class="mw-page-title-main">Jennifer Webster-Cyriaque</span> American dentist and immunologist

Jennifer Y. Webster-Cyriaque is an American dentist and immunologist specializing in the oral microbiome, salivary gland disease in patients with HIV, and cancer-causing viruses. She became the deputy director of the National Institute of Dental and Craniofacial Research in November 2020. Webster-Cyriaque was a faculty member at UNC Adams School of Dentistry and the UNC School of Medicine for 21 years.

References

  1. ↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN   978-0-7216-2921-6.
  2. ↑ Cawson RA, Odell EW, Porter S (2002). Cawsonś essentials of oral pathology and oral medicine (7th ed.). Edinburgh: Churchill Livingstone. pp. 223, 224. ISBN   978-0443071065.
  3. ↑ "Leukoplakia". The Lecturio Medical Concept Library. Retrieved 1 August 2021.
  4. 1 2 3 Cherry-Peppers, G; Daniels, CO; Meeks, V; Sanders, CF; Reznik, D (February 2003). "Oral manifestations in the era of HAART". Journal of the National Medical Association. 95 (2 Suppl 2): 21S–32S. PMC   2568277 . PMID   12656429.
  5. 1 2 3 4 5 6 7 8 9 10 11 12 Neville BW, Damm DD, Allen CM, Bouquot JE (2002). Oral & maxillofacial pathology (2nd ed.). Philadelphia: W.B. Saunders. pp.  241–242. ISBN   978-0721690032.
  6. ↑ Gulley, ML (February 2001). "Molecular diagnosis of Epstein-Barr virus-related diseases". The Journal of Molecular Diagnostics. 3 (1): 1–10. doi:10.1016/s1525-1578(10)60642-3. PMC   1907346 . PMID   11227065.
  7. 1 2 3 4 5 6 Scully C (2008). Oral and maxillofacial medicine : the basis of diagnosis and treatment (2nd ed.). Edinburgh: Churchill Livingstone. pp. 216, 308, 310–312. ISBN   9780443068188.
  8. 1 2 3 4 5 6 7 Chapple, IL; Hamburger, J (August 2000). "The significance of oral health in HIV disease". Sexually Transmitted Infections. 76 (4): 236–43. doi:10.1136/sti.76.4.236. PMC   1744197 . PMID   11026876.
  9. ↑ EC Clearinghouse. Oral Problems Related to HIV Infection, revised classification.
  10. ↑ Jung, AC; Paauw, DS (February 1998). "Diagnosing HIV-related disease: using the CD4 count as a guide". Journal of General Internal Medicine. 13 (2): 131–6. doi:10.1046/j.1525-1497.1998.00031.x. PMC   1496917 . PMID   9502375.
  11. ↑ "Pioneering AIDS Researcher Receives Major Accolade | UC San Francisco". April 15, 2022. Archived from the original on 15 April 2022.
  12. ↑ Greenspan, D; Greenspan, JS; Conant, M; Petersen, V; Silverman S Jr; de Souza, Y (Oct 13, 1984). "Oral "hairy" leucoplakia in male homosexuals: evidence of association with both papillomavirus and a herpes-group virus". Lancet. 2 (8407): 831–4. doi:10.1016/s0140-6736(84)90872-9. PMID   6148571. S2CID   7928436.
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