2010.2-4.Focal

                                                                                                                            article in PDF  
N Dermatol Online. 2010; 1(2): 26-28
Conflicts of interest: None
 

FOCAL EPITHELIAL HYPERPLASIA: HECK’S DISEASE

FOCAL EPITHELIAL HYPERPLASIA: CHOROBA HECK’A

Piotr Brzeziński

6th Military Support Unit, Ustka, Poland
 

Corresponding author: Dr. Piotr Brzeziński    e-mail: brzezoo77@yahoo.com


 

Abstract
Heck's disease (focal epithelial hyperplasia) (FEH) is clinically characterized by multiple circumscribed, soft elevated nodules of the oral mucosa. Papules and plaques are usually the color of normal mucosa, but may be pale or, rarely, white. Disease may persist for years, producing a significant reduction in quality of life. Lessions may be located in the area of mucous membranes of the lips, cheeks and tongue. It is believed that the two virus types are responsible for the FEH, are: HPV 13 and HPV 32. Different therapeutic procedures have been reported: surgical excision, laser ablation, cryotherapy, electrocauterization, interferon, retinoic acid, 5% immiquimod.
 
Streszczenie
Choroba Heck’a (focal epithelial hyperplasia -FEH) klinicznie charakteryzuje się wieloma ograniczonymi, miękkimi grudkami zlokalizowanymi na błonie śluzowej jamy ustnej. Grudki i tarczki są zwykle koloru normalnej błony śluzowej, ale mogą być blade lub rzadziej białe. Choroba może trwać przez wiele lat, powodując znaczne obniżenie jakości życia. Zmiany mogą być umiejscowione w okolicy błon śluzowych warg, policzków oraz języka. Uważa się, że są dwa typy wirusów odpowiedzialnych za FEH: HPV 13 i HPV 32. W leczeniu FEH stosowano leczenie chirurgiczne, ablację laserową, krioterapię, elektrokauteryzację, interferon, kwas retinowy, 5% krem imikwimod.
 
Key words:  Focal epithelial hyperplasia; Heck's disease; human papilloma virus (HPV)
Słowa klucze:  Focal epithelial hyperplasia; Choroba Heck'a; human papilloma virus (HPV)

 

Heck's disease, which is also known as "focal epithelial hyperplasia" and "hyperplasia multilocularis mucosae oris", was first described in 1965 by Archard et al [1]. Heck's disease (focal epithelial hyperplasia) (FEH) is clinically characterized by multiple circumscribed, soft elevated nodules of the oral mucosa. Papules and plaques are usually the color of normal mucosa, but may be pale or, rarely, white. Hyperplastic lesions are small (0.3-1.0 cm.). Disease may persist for years, producing a significant reduction in quality of life. Lessions may be located in the area of mucous membranes of the lips, cheeks and tongue. The disease affects both sexes and all ages, but is seen predominantly in children [2]. FEH has been reported especially in Indians and Eskimos. FEH in a few cases reported in Caucasians [3,4]. In Poland the disease is rare. Analysis of the literature shows only one studies [4]. This prevalence of the disease seems to be of genetic predisposition [5]. In many studies it has been shown that malnutrition, poor hygiene, and social conditions have also been associated with this disease [2]. The diagnosis of FEH can be made clinically but histological examination may show characteristics of viral infection, as in our case. However, the precise diagnosis is made by in situ hybridization or PCR analysis. It is believed that the two virus types are responsible for the FEH, are: HPV 13 and HPV 32 [5,6,7], in rare cases HPV 1 and 18 were demonstrated [8]. FEH is somewhat different from other HPV infections in that it is able to produce extreme paraceratosis, acanthosis or hyperplasia of the prickle cell layer of the epithelium. The mucosa may be 8-10 times thicker than norma [1]. The course o the disease is with mild and a spontaneous remission is possible. Niebrügge et all report on the rare and unusual case of Heck's disease in a 64-year-old European woman with an 24 HPV-associated malignant transformation [9]. The differential diagnosis FEH should be made against condyloma acuminatum, florid oral papillomatosis, Cowden’s syndrome, Crohn’s disease, Cannon’s disease (White Sponge Nevus) or Gorlin-Goltz syndrome (Focal Dermal Hypoplasia [10,11]. Different therapeutic procedures have been reported (surgical excision, laser ablation, cryotherapy, electrocauterization, interferon, retinoic acid) and CO2 laser surgery seems to be one of the best [12]. Advantages of this method are the haemostatic effect, the healing without scarring and the compliance of the patient. For 17-year-old boy described by Greek authors, relapses were not observed for 20 months after laser treatment [3]. Pedro Ponte et al show beneficial effects of treatment FEH with 5% cream Imiqouomid in two siblings [13]. Similar observations represent Turkish [14] and Deutsch authors [15]. Yasar et al observed no complications or recurrence one year after treatment imiquomidem. However, Mischke et al used the 5% imiquimod cream was applied 3 times per week. Regression of lesions was obvious after 1 month of treatment. Complete clearance was achieved after 2 additional months of treatment and no recurrence was detected over a follow-up period of 5 months. Alakloby OM and M Akram Randhawa from Saudi Arabia, observed in the course of resolution of the FEH after application acitretin [10]. Acitretin (Neotigason) 0.5 mg/kg/day was advised orally and patient asked to report after one month. There was a little improvement after the first month. A favourable improvement was observed after two months.
 
REFERENCES
1. Archard HO., Heck JW., Stanley HR.: Focal epithelial hyperplasia: an unusual oral mucosal lesion found in Indian children. Am Acad Oral Pathol 1965; 20: 201-212. 2. Hashemipour MA., Shoryabi A., Adhami S., Honarmand HM.: Extensive Focal Epithelial Hyperplasia. Arch Iran Med 2010; 13: 48-52 3. Bassioukas K., Danielides V., Georgiou I., Photos E., Zagorianakou P., Skevas A: Oral focal epithelial hyperplasia.Eur J Dermatol. 2000; 10: 395-397. 4. Majewski S., Jablonska S.: Skin autografts in epidermodysplasia verruciformis human papilloma virus associated cutaneous changes need over 20 years for malignant conversion. Cancer Resarch 1997; 57: 4214- 4216. 5. Binder B., Wieland U., Smolle J.: Focal epithelial hyperplasia (Heck disease) in a black child.Pediatr Dermatol. 2007; 24: E31-32. 6. Petzold D., Dennin R.: Isolierung virus-artigen Partikeln bei fokalen epithelialer Hyperplasie Heck. Hautartz 1980; 31: 35-36. 7. Williamson AL., Denis SL.: The use of the polymerase chain reaction for the detection of human papilloma virus type 13. J Virol Methods 1991; 31: 57-65. 8. Lang E., Zabel M., Ikenberg H.: Fokale epitheliale Hyperplasie. Dt Med Wochenschr 1984; 109: 1763-1766. 9. Niebrügge B., Villiers E., Gerlach K., Franke I., Gollnick H.: Demonstration of HPV 24 in long-standing Heck's disease with malignant transformation.Eur J Dermatol. 1999; 9: 477-479 10. Saint-Gerons RS., Rojas MT., Salobreńa AC., Soria JLA., Vaamonde HF.: Hiperplasia epitelial focal. Una rara enfermedad en nuestro medio. Med Oral Patol Oral Cir Bucal. 2005; 10: 128-131. 11. http:// panarabderm.org/en/components/com_medi/ upload/PALD_vol20_1_52-55.pdf 12. Carruth JAS.: Resection of the tongue with the carbon dioxide laser. J Larygol Otol 1982; 96: 529-543. 13. Ponte P., Serrão V., Fiadeiro T.: Focal epithelial hyperplasia (Heck’s Disease) in two siblings: response to treatment with imiquimod 5% cream. Eur J Dermatol. 2010; 20: 248-249 14. Yasar S., Mansur AT., Serdar ZA., Goktay F., Aslan C.: Treatment of focal epithelial hyperplasia with topical imiquimod: report of three cases. Pediatr Dermatol. 2009; 26: 465-458. 15. Maschke J., Brauns TC., Goos M.: Imiquimod for the topical treatment of focal epithelial hyperplasia (Heck disease) in a child. J Dtsch Dermatol Ges. 2004; 2: 848-850


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