Heck's Disease Research Articles

Prevalence of herpesviruses in Yanomami indigenous people and its relationship with Heck's disease.

Prevalence of herpesviruses in Yanomami indigenous people and its relationship with Heck's disease.

Natural History of Oral HPV Infection among Indigenous South Australians.

This study aims to describe the natural history of and identify the risk factors associated with oral human papillomavirus (HPV) infections in an Australian Indigenous cohort. A longitudinal cohort study design, with baseline (2018), 12-month, and 24-month data obtained from Indigenous Australians aged 18+ years in South Australia, was performed. Face-to-face interviews were conducted, and saliva samples for HPV testing were collected at each time point. Basic descriptive analyses were conducted to calculate prevalence, incidence, persistence, clearance, and incidence proportions of any HPV infection. Multivariable logistic regression analyses with adjusted prevalence ratios (PRs) were conducted to identify risk factors associated with oral HPV infection. Among 993 participants with valid saliva samples, 44 HPV types were identified. The prevalence of infection with any oral HPV infection was 51.3%, high-risk HPV was 11%, and types implicated in Heck's disease (HPV 13 or 32) was 37.4%. The incidence, persistence, and clearance of any and high-risk HPV infections were 30.7%, 11.8% and 33.3% vs. 9.3%, 2.8%, and 9%, respectively. Our findings indicate that the prevalence, incidence, and persistence of oral HPV infection in a large sample of Indigenous Australians were high, and clearance was low. Oral sex behaviours and recreational drug use were risk factors associated with incident high-risk HPV infection.

A severe manifestation of extensive multifocal epithelial hyperplasia (Heck's disease) and the role of CO2 laser as a treatment modality: A case report

AbstractHeck's disease or multifocal epithelial hyperplasia (MEH) is an uncommon asymptomatic contagious oral condition, usually attributed to human papillomavirus (HPV) infection. It manifests as several smooth papules and nodules in the oral mucosa. The disease has a good prognosis, as it is spontaneously regressing, but some cases persist and affect the quality of the patient's life, requiring therapeutic intervention. We present an unusual case of extensive multifocal epithelial hyperplasia. To the best of our knowledge, this is the first case to be reported with this severity and widespreadness of the lesions. A 14‐ year‐old girl was referred to the department of oral medicine at the faculty of dental medicine, with a chief complaint of numerous nodules and papules, appeared since she was 6 years old. The diagnosis of Heck's disease was confirmed depending on clinical characteristics, histopathological examination and immunohistochemical (IHC) staining. Human immunodeficiency virus (HIV) antibodies test and enzyme‐linked immunosorbent assay (ELISA) for human papillomavirus Immunoglobulin G (IgG) were performed. Depending on the extensive clinical situation, the decision was made to treat the patient using carbon dioxide (CO2) laser. Heck's disease is a human papillomavirus‐related oral condition. The diagnosis can be confirmed depending on both clinical and histopathological characteristics in addition to immunohistochemical staining. In this case, CO2 laser showed good functional and aesthetic results that reflected positively on the quality of the patient's life.

FOCAL EPITHELIAL HYPERPLASIA (HECK'S DISEASE) IN AN ADULT PATIENT: CASE REPORT

Focal epithelial hyperplasia, or Heck's disease, is a rare benign lesion of the oral mucosa. It is usually associated with the proliferation of the squamous epithelium mainly attributed to human papillomavirus (HPV). We report the case of a 38-year-old dark-skinned man who attended the health service of the university complaining of an oral mucosa burning sensation. On clinical examination, the patient presented multiple pink papular lesions, firm by palpation, with agglomerated or isolated papules located on the alveolar ridge, inserted and free gingiva of the upper and lower arch, and buccal and labial mucous membranes consistent with HPV lesions. Focal epithelial hyperplasia was the clinical diagnosis. An incisional biopsy of the oral lesions was performed, and the histopathology report described the fragment of oral mucosa covered by stratified parakeratinized squamous epithelium with prominent acanthosis and confluent focal projections to the connective tissue, corroborating the previous clinical diagnostic suspicion.

Fully edentulous patient with multifocal epithelial hyperplasia: Report of very rare case

Multifocal epithelial hyperplasia (MEH), also known as verrucae of the oral cavity, focal epithelial hyperplasia, Heck's disease, and multifocal papillomavirus epithelial hyperplasia, is a rare benign oral condition. It is most typically seen in the labial mucosa of younger people and rarely seen in middle-aged patients. Multiple small nodules or papules in the oral cavity, notably on the labial mucosa, buccal mucosa, and tongue, are clinically defined by this lesion. For precise diagnosis needs, clinical history, examination, and histopathologic findings. After months or years, spontaneous regression was commonly recorded; therefore, no treatment was required. Conservative surgical excision can be used for diagnostic and aesthetic reasons and recurrent trauma lesions. We present a very rare oral multifocal epithelial hyperplasia case involving the generalized attached gingiva of the upper and lower jaw in a fully edentulous patient in the Oral Specialized Clinic, Tehran, Iran.

Useful cytological confirmation of HPV 13 in lesional mucosa enhances diagnosis of focal epithelial hyperplasia

An 11-year-old female presented with multiple oral lesions for several months. Histopathological findings suggested focal epithelial hyperplasia (FEH), also known as Heck disease. FEH is strongly associated with Human papillomavirus (HPV), especially genotypes 13 and 32. An oral swab of a mucosal lesion was subsequently obtained for cytology, immunohistochemistry and in situ hybridization. In addition, in situ hybridization and immunohistochemistry were also performed retrospectively on the biopsy specimen for correlation. The cytology specimen showed squamous cells with enlarged, slightly atypical nuclei and rare perinuclear halos. The histology findings included papillomatosis with acanthosis, mild nuclear atypia and focal perinuclear halos. The immunohistochemistry for the consensus HPV L1 capsid protein was found in both the cytology and biopsy specimens indicating that the lesion was HPV-related. High viral copy numbers of HPV 13 were detected by in situ hybridization in both the cytology and histology specimens. Although histologic features of FEH have been well characterized in the literature, to our knowledge, this is the first case to describe in FEH with adjunct immunohistochemistry and in situ hybridization results. Furthermore, these findings assisted in our diagnosis since the patient's clinical presentation was a diagnostic challenge with smooth dome-shaped papules instead of the typically described flat-topped verrucous lesions seen in FEH. In summary, our case reveals that there is a high concordance between the HPV 13 detection in the cytology and histology of FEH, and that performing cytology in addition to histology can be used to optimize diagnostic evaluation towards appropriate patient care.

Refractory focal epithelial hyperplasia successfully treated with novel use of nano-pulse stimulation technology.

Focal epithelial hyperplasia (FEH), or Heck's disease, is an uncommon HPV-related dermatosis that presents with multiple discrete papules or nodules on the lips, tongue, and gingival or oral mucosa. Treatment is often sought due to cosmesis, social stigma, and functional impairment. Treatment is challenging and a variety of treatment modalities have been attempted with varying degrees of success. This report describes the novel use of nano-pulse stimulation in the successful treatment of recalcitrant FEH of the upper and lower vermillion lip.

CHANGING TRENDS IN BENIGN HUMAN PAPILLOMA VIRUS (HPV) RELATED EPITHELIAL NEOPLASMS OF THE ORAL CAVITY: 1995-2015

<h3>Introduction</h3> HPV-related benign papillary epithelial neoplasms are common lesions affecting any region of the oral cavity. This study evaluated the trends in frequency, location, and demographics of these lesions over 20 years. <h3>Materials and Methods</h3> Following IRB approval, the archives of UF Oral Pathology Biopsy Service between 19952015 were queried. Squamous papilloma, verruca vulgaris, and viral papilloma/condyloma were included. Extraoral locations, inconclusive diagnoses, or syndrome-related HPV lesions (Heck disease) were excluded. Age, gender, location, clinical presentation, and diagnoses were recorded. Data from one calendar year per 5-year span was assessed including the years 1995, 2000, 2005, 2010, and 2015. <h3>Results</h3> A total of 1458 cases were identified over the total 5 calendar years assessed. Papilloma as a percentage of total biopsies per year was as follows: 1995 (2.6%), 2000 (3.3%), 2005 (3.6%), 2010 (4%) and 2015 (4.5%), representing a 73% (1.9x) percentage increase. Males (56%) were affected more commonly; however, in patients under 19 years, a female predominance was observed. The overall percentage of lesions in females increased by 30.6% over the time frame. Mean age was 54 years (range 1-93 years) and increased by 10 years over time. About 1.1% of patients had multifocal lesions and 0.2% had a recurrence. In descending order of frequency, tongue, soft palate, and mandibular gingiva were most involved. Maxillary gingiva and lower lip were the most common locations in patients under 19. Location varied over time with the biggest increase noted on the gingiva. Squamous papilloma was the most common histologic variant (93.6%). <h3>Conclusion</h3> The incidence of benign HPV-related oral lesions increased substantially over the 2 decades studied. Other trends noted included increase in the following: average age of patients, female involvement, and gingival location. Our results likely indicate an overall increase in the prevalence of benign oral HPV lesions in our population.

Focal epithelial hyperplasia (Heck's disease) in Brazilian indigenous children

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An Unusual Observation of Oral Multifocal Epithelial Hyperplasia in Elderly Man

Multifocal epithelial hyperplasia or Heck's disease is uncommon, contagious, asymptomatic, and benign disease affecting the oral mucosa caused by some subtypes of human papilloma virus, especially subtypes 13 or 32. It affects mostly pediatric patients and adolescents in certain geographical regions and ethnic groups. Clinically, the lesion is characterized by the presence of multiple soft papules or nodules in the oral cavity. This paper reports a Tunisian case of 52-year-old man presented with Heck’s disease. The diagnosis is based on both clinical aspect and histopathological features. Clinicians should be familiar with this rare oral disease, which can even affect elderly patients as reported in our case. Treatment is not mandatory and may be indicated when there is esthetic and/or functional impairment.

HECK'S DISEASE – A RARE CASE REPORT

Heck's disease is a familial benign lesion affecting the oral mucosa and skin caused by Human papilloma viruses 13 and 32. This condition is quite rare in the Indian subcontinent and till date less than 10 case reports have been published. Females are predominantly affected. It is important for an oral physician to be aware and possess sufcient knowledge about these lesions as these lesions are highly contagious. Further, the presence of these lesions may indicate an underlying immunodecient state of the patient. This paper reports an unusual case of Heck's disease in a 30 year old Indian male.

Heck’s disease occurring after Epstein‐Barr virus‐associated smooth muscle tumors in an immunosuppressed child

A 10-year-old Guatemalan girl with past medical history of Epstein-Barr virus-associated smooth muscle tumors (EBV-SMT) and combined immunodeficiency presented for evaluation of painful intraoral lesions. On examination, she was noted to have multiple, white to flesh-colored, soft, flat-topped papules, and plaques on the buccal and labial mucosa. Human papillomavirus type 13 was detected on PCR with PGMY primers of previously biopsied buccal tissue, confirming a diagnosis of Heck's disease (multifocal epithelial hyperplasia). We present an immunosuppressed, pediatric patient with two rare, virus-associated neoplastic disorders that have not been previously reported to occur in the same individual.

Prevalence of Oral Human Papillomavirus Infection Among Australian Indigenous Adults

Human papillomavirus (HPV) infection is associated with oropharyngeal squamous cell carcinoma. International estimates suggest overall oral HPV prevalence is 7.5%, with prevalence of oral HPV types 16 and 18 being 1.6%; prior Australian estimates suggest oral HPV prevalence is 2.3%, with HPV-16 and HPV-18 being 1.3%. To estimate the prevalence of oral HPV infection among Indigenous Australians and to report the prevalence of factors associated with high-risk HPV types (ie, HPV-16 and HPV-18) and HPV types linked with Heck disease (ie, HPV-13 and HPV-32). This cross-sectional study analyzed HPV screening results from saliva samples collected from 1011 Indigenous Australians between February 2018 and January 2019. Data were analyzed from May 2018 to May 2019. Recruitment occurred through Aboriginal Community Controlled Health Organisations in South Australia. Eligibility included identifying as Indigenous, residing in South Australia, and being aged 18 years or older. Saliva samples were collected, with microbial DNA for genotyping extracted. Sociodemographic parameters, health-related behaviors, and sexual history data were collected. Analyses were stratified by sex as well as by HPV types 13 and 32 (Heck disease) and 16 and 18 (high risk of oropharyngeal squamous cell carcinoma). Multivariable analyses were conducted to obtain adjusted odds ratios (ORs). Data were obtained for 910 participants (median [interquartile range] age, 37 [27-51] years); 595 participants (65%) were female and 572 (63%) resided in nonmetropolitan locations. In all, 321 saliva samples (35.3%; 95% CI, 32.2%-38.4%) were positive for oral HPV (106 [33.7%] men; 215 [36.1%] women). The highest prevalence was found for HPV types 13 and 32 (207 [22.7%] total; 60 [19.0%] men; 147 [24.7%] women) followed by HPV types 16 and 18 (30 [3.3%] total; 9 [2.9%] men; 21 [3.5%] women). After multivariable analysis, risk factors associated with HPV types 13 and 32 included nonmetropolitan residential status (OR, 2.06; 95% CI, 1.10-3.88) and not having had a tonsillectomy (OR, 2.74; 95% CI, 1.05-7.16). Among women, having obtained a high school education or less was associated with lower odds of HPV-16 and HPV-18 infection (OR, 0.16; 95% CI, 0.03-0.97). Prevalence of oral HPV infection in a large sample of Indigenous Australians was high, with one-third testing positive. The most prevalent HPV types were those associated with Heck disease. The prevalence of HPV types associated with oropharyngeal squamous cell carcinoma exceeded both Australian and international population-level estimates.

HECK DISEASE IN A 7-YEAR-OLD AMAZONIC BOY: A CASE REPORT

A 7-year-old male patient was referred to a dentistry specialty center because of several papillary lesions of the same color as the surrounding mucosa, which were painless and nonbleeding in the inner cheek and labial mucosa. The diagnosis hypothesis was multifocal epithelial hyperplasia (Heck disease). An incisional biopsy was performed, and the histopathologic findings in stratified squamous epithelium were parakeratosis, acanthosis, and thick epithelial rete ridges, sometimes being club-shaped. The epithelial cells presented nuclei surrounded by clear halos, mitotic figures, and mitosoid cells. The underlying connective tissue presented scarce inflammatory cells. The data confirmed the original clinical hypothesis of multifocal epithelial hyperplasia (Heck disease). No treatment was performed because of the self-limited characteristics of the disease. The patient is now under 6-month follow-up, already showing early signs of lesion remission.

High Prevalence of Oral HPV Infection Among Indigenous Australians: An Observational Study

Background: Human papillomavirus (HPV) infection is casually associated with oropharyngeal squamous cell carcinoma (OPSCC). International estimates suggest overall oral HPV prevalence is 7.5% (95% CI 9.7-8.4), with prevalence of oral HPV16/18 being 1.6% (95% CI 1.2-2.1). Prior Australian estimates suggest oral HPV prevalence is 2.3% (95% CI 0.6-3.9), with HPV16/18 being 1.3% (95% CI 0.0-2.6). Heck's disease is a benign condition caused by HPV types 13/32. The study aimed to estimate: (1) oral HPV prevalence among Indigenous Australians; (2) risk factors associated with high risk HPV types and; (3) risk factors associated with HPV types linked with Heck's disease. Methodas: Eligibility included identifying as Indigenous, residing in South Australia and being aged 18+ years. Saliva samples were collected, with microbial DNA for genotyping extracted. Socio-demographic parameters, health-related behaviours and sexual history data were collected. Analyses were stratified by sex, and by HPV types 13/32 (Heck's disease) and 16/18 (high risk for OPSCC). Findings: Data were obtained for 910 participants recruited between Feb 2018 and Jan 2019, age range 18-82 years, median age 37 years. Around one-third (35.3%, 95% CI 32.2-38.4) of saliva samples were positive for oral HPV (33.7% male, 36.1% female). The highest prevalence were HPV types 13/32 (22.7%; 19.0% male, 24.7% female) followed by HPV types 16/18 (3.3%; 2.9% male, 3.5% female). Associations with HPV types 13/32 included non-metropolitan residential status, low socioeconomic status, tonsillectomy, not having given oral sex, not having received oral sex or having sex with less than 4 people. Residing in a metropolitan location was associated with HPV types 16/18. Interpretation: Prevalence of oral HPV in a large sample of Indigenous Australians was high, with one-third testing positive. The most prevalent HPV types were those associated with Heck's disease. The prevalence of HPV types associated with OPSCC exceeded both Australian and international population-level estimates. Funding Statement: National Health and Medical Research Council APP1120215. Declaration of Interests: KC is co-PI of an investigator-initiated trial of primary HPV screening in Australia (‘Compass’) which is conducted and funded by the Victorian Cytology Service, a government-funded health promotion charity, which has received a funding contribution from Roche Molecular Systems, CA and Ventana Inc., USA, but neither KC nor her institution on her behalf have received funding from industry for this or any other project. All other authors declare no competing interests Ethical Approval Statement: Ethical approval was received from the University of Adelaide Human Research Ethics Committee and the Aboriginal Health Council of South Australia’s Human Research Ethics Committee. Participants signed informed consent forms.

The molecular-based differentiation of Heck's disease from its mimics including oral condyloma and white sponge nevus

Heck's disease (focal or multifocal epithelial hyperplasia) is a benign, rare condition of the skin and mucous membranes induced by human papillomavirus (HPV) infection. Other entities that can induce large papillomatous lesions that involve the mucous membranes and skin include condyloma acuminatum, which is sexually transmitted, and white sponge nevus, often due to a mutation of cytokeratin 4 or 13. Six cases diagnosed as either Heck's disease (n = 2) or white sponge nevus (n = 4) and 6 oral condyloma were compared on histologic grounds and analyzed in situ for HPV DNA, including HPVs 6,11, and 13, as well as cytokeratins 4 and 13. Each case showed marked acanthosis, and para/hyperkeratosis. More variable histologic findings included rete ridge elongation, keratinocyte degeneration, and perinuclear halos. High copy HPV 13 DNA was evident in the squamous cells towards the surface in the two cases diagnosed as Heck's disease and in two cases diagnosed as white sponge nevus on clinical grounds. HPV 6/11 was found in each of the six condyloma. Marked decrease in either cytokeratin 4 or 13 was evident in the two cases diagnosed as white sponge nevus that were HPV DNA negative. It is concluded that in situ hybridization analyses including HPVs 6, 11, and 13 as well as immunohistochemistry for cytokeratins 4 and 13 can differentiate Heck's disease from condyloma and white sponge nevus, which can be difficult to differentiate on clinical and histologic grounds.

Heck's Disease Treated with CO2 Laser: a Case Report

Multifocal epithelial hyperplasia or Heck's disease is a rare viral infection of the oral mucosa caused by human papillomavirus types 13 and 32. It is characterized by multiple painless sessile papules that can coalesce to form larger lesions such as plaques or nodules. A 53-year-old woman presented with multiple sessile papules or nodules in the tongue and reported a sensation of discomfort. The lesions were similar in color to the normal mucosa. An incisional biopsy was performed with the hypothesis of multifocal epithelial hyperplasia and the histological analysis showed acanthosis and epithelial papillomatosis. The clinical and histopathological findings were compatible with the diagnosis of Heck's disease. The treatment was performed, for functional reasons, with ablation using a carbon dioxide laser (CO2). The patient reported postoperative well-being, regression of lesions, and improvement of functionality.

Focal Epithelial Hyperplasia (Heck's Disease): an Unexpected Finding

Focal Epithelial Hyperplasia (Heck's Disease): an Unexpected Finding

A Case Report of Multifocal Epithelial Hyperplasia (Heck's Disease) Treated with CO2 Laser

Focal epithelial hyperplasia or Heck’s disease is a rare benign lesion of oral mucosa caused by human papilloma virus especially subtypes 13 and 32. The disease is predominantly found in children and adolescents with indigenous heritage. This paper reports a unique case of Heck's disease in an 11-year-old Iranian girl with multiple, bilateral and elevated lesions on the buccal and labial mucosa. The lesions had persisted for a year. The disease was diagnosed as focal epithelial hyperplasia through the clinical examination. Considering esthetic and functional problems, CO2 laser (Wavelength: 10600 nm) surgery was determined as a suitable choice of treatment. The patient was followed up over 1 year with no signs of lesion recurrence.

Skin diseases in indigenous population: retrospective epidemiological study at Xingu Indigenous Park (XIP) and review of the literature.

Skin diseases among indigenous populations have been poorly described in the literature. Risk factors linked to habits, cultural practices, genetics, and environmental characteristics can influence the frequency and clinical presentation of these diseases. This was a cross-sectional study conducted through a review of dermatological medical records. Data were obtained over three visits to Xingu Indigenous Park (XIP). Main dermatoses were compared by gender, age, ethnicity, and village. Diseases were classified into four groups: infectious, inflammatory, neoplastic, and other. Of the 398 patients included in the study, 54.2% were female. The participants ranged in age from 1 to 75 years (median, 10 years). The predominant ethnic group was Caiabi (49.7%), followed by Ikpeng (14.3%) and Kamaiurá (10.0%). The primary village studied was Diauarum, followed by Moigú and Morena. Infectious dermatoses were the most frequent (43.8%), followed by inflammatory (33.7%), neoplastic (15.5%), and other skin diseases (7.0%). Infectious diseases were predominant in Caiabi and Kamaiurá Indians, whereas inflammatory skin diseases were more frequent in Ikpeng and Juruna populations. We also observed a high frequency of malignancy in the Ikpeng and Trumai ethnic groups. Infectious skin diseases, except for fungal infections, were frequent in younger age groups. This trend was not replicated for neoplastic skin diseases. Uncommon dermatoses such as Heck's disease, lichen striatus, and aquagenic urticaria were also observed. Jorge Lobo's disease was not included in this analysis. Improved knowledge of the distribution of common dermatoses among different ethnic groups and villages in XIP will aid in the prevention of skin infections and skin cancer. This improved knowledge will also allow the development of skin disease guidelines that can help local indigenous health professionals.