August 1992, Volume 42, Issue 8

Case Reports

FOCAL EPITHELIAL HYPERPIASIA A NEWLY DISCOVERED DISEASE IN NORTH WEST FRONTIER PROVINCE OF PAKISTAN

Inam Ullah Khan  ( Armed Forces Institute of Pathology, Rawalpindi. )
Manzoor Ahmed  ( Armed Forces Institute of Pathology, Rawalpindi. )
Irshad Hakim  ( Dermatology Department, Postgraduate Medical institute, Lady Reading Hospital, Peshawar. )
M. Mumtaz Khan  ( Armed Forces Institute of Pathology, Rawalpindi. )

INTRODUCTION

The term focal epithelial hyperplasia was intro­duced in 1965 to describe certain multiple nodular elevations of the oral mucosa observed among American Indians in New Mexico, USA and Mato Grossa district in Brazil1. Since then it has been reported from several other countries including Iraq, Israel and South Africa2. We report two cases of this disorder from North Western Frontier Province of Pakistan.

CASE 1
An Afghan boy, aged 10 years, reported to the dermatology unit of Postgraduate Medical Institute, Lady Reading Hospital, Peshawar with complaints of asymptomatic multiple nodular lesions in the oral mucosa (Figure 1).

He was referred from orodental department for evaluation of any systemic cause of his illness. The father had noticed a few nodular elevations on the lower lip of the child 5 years ago, which had progressively increased in size and number. New lesions started appearing on the upper lip, sides of the tongue, hard palate and other areas of the oral mucosa. The past and family history were not contributory. On clinical examination numerous rounded to polygonal fleshy elevations were present on labial mucosa, sides of the tongue, at the junction between hard and soft palate and hard palate itself. Their size ranged from 0.1 to 0.5 cm in diameter. They were soft to firm in consistency and nontender. Indirect laryngos­copy was normal. All the relevant laboratory investigations, i.e., com­plete blood picture, urinalysis, chest x-ray, thyroid function tests and scan were within normal limits. VDRL test• was negative. A biopsy was taken which showed epithelial hyperplasia with parakeratosis, acanthosis, papillomatosis, elongation and anastomosis of rete ridges (Figure 2).

A diagnosis of focal epithelial hyper. plasia was made. The lesions were treated with cryosurgery in four different sittings at two weeks intervals. The response to this modality was good and the lesions did not recur until! 6 months of follow- up.
CASE 2
A ten year old female child from Chitral reported with asymptomatic papulonodular elevations on the upper and lower lips causing cosmetic concern. A few papular elevations on the lower lip of the child were noted 2 years before which subsequently increased in number and size and also started appearing on the upper lip. No family history of such problem was present. On clinical examination numerous soft to firm, white to flesh coloured papules were seen on the lower and upper labial mucosa. A few scattered lesions were also present on the tongue and other parts of the oral mucosawhose size ranged from 0.1 to 0.5cm in diameter. The lesions on the lower lip were somewhat whitish and slightly hyperkeratotic. They were less noticeable on stretching the oral mucosa. Indirect Iaryngoscopy did not reveal any such lesions in the larynx. All the relevant investigations were within normal limits. Histology of an qral papule showed epithelial hyperplasia with marked acanthosis, papillomatosis, elongatton and tusion 01 me rcte ridges. Some vacuolated cells were also present in the epidermis. A diagnosis of focal epithelial hyperplasia was made. The lesions on the lower lip were treated with cryosurgery but the patient was lost to follow-up.

DISCUSSION

The first complete description of focal epithelial hyperplasia was made by Archard, Heck and Stanley3 who, described the condition as nodular masses on the oral mucosa usually from 1 to 5 mm in diameter and often so numerous that they appear sprinkled over the mucosal surfaces. The nodules are soft and elevated with surface like the adjoining oral mucosa or have a flat whitish surface1. This condition predominates in children and young adults, 3 to 18 years of age. The most commonly reported site is the lower labial mucosa. The floor of the mouth and palatal mucosa are conspicuous for their absence of lesions4. Both of our cases fulfil these criteria except that our first case had involvement of the hard palate also. Familial occurrence of this disease has been described5 but no such association was found here. The focal epithelial hyperplasia occurs with an unusual racial and geographic distribution appearing in 30% of the Eskimos of Greenland and in 3% of the Indians of North, Central and South America. it rarely occurs in the rest of the world population6 which could be in part due to ignorance among clinicians about this newly described entity. It may be useful to carry out a survey to determine the prevalence of this condition in our population. There has been convincing recent evidence now that a type of human papillorna virus may be the major factor in the causation of this disease6-8. Papova virus group particles have been identified by electron micros­copy8 and immunoperoxidase techniques6. More recent studies with DNA hybridization techniques suggest UPV 139,10 and HPV 3210 to be associated with focal epithelial hyperplasia. A high risk HPV type 16 DNA11 has also been found in one case of focal epithelial hyperplasia. The negative family history and the clinical course of the disease in both of our cases support this notion, however the possibility of genetic factors in its causation cannot be ruled out1,5,6. The disease is said to regress spontaneous­ly2. This does not appear to be the case in our patients as both of them had a long history of slowly progressive disease. The possibility that the disease may have a different course in our patients cannot therefore, be ruled Out. The main morphological changes described so far arc epithelial hyperplasia, parakeratosis, acanthosis, elongation and anastomosis of rete ridges1,7,12. Dys­keratosis, epithelial atypia and inclusion bodies have not been found9. Our histopathological findings correlate well with those described. However, basal liquefaction (an occasional finding9) was not seen in our cases. The treatment modalities described are electrocoagulation, excision and shaving4,6. Cryosurgery has been described to be ineffective4. It appeared to be very effective in our first case. More studies, however, are needed to determine the effectiveness of this form of treatment.

REFERENCES

1. Pinborg. J.J. Disorders of the oral cavity and lips, in textbook of dermatology. Edited by Aurthur Rook et at. 4th ed. Oxford, Blackwell, p. 2084.
2. Vanwyk, c.w., Staz, J. and Farman, A.G. Focal epitheliat hyperplasia in a groupofSouth Africans; its clinical and microscopic features. 3. Oral. Pathol., 1977; 6:1-13.
3. van Wyk, C.W. Focal epithelial hyperplasia of the mouth: recently discovered in South Africa. Br.J. Dermatol., 1977;96:381-88.
4. Stiefler, RE., Solomon, M.P. and Shalita, AR. Heck’s disease (focal epithelial hyperplasia). J.Am.Acad. Dermatol., 1979;1:499- 503.
5. Gomez, A, Calle. C., Arcila, G., et al. Focal epithelial hyperplasis in a half-breed family of Columbians. J.Am.Dent. Aaaoc., 1969;79:663-67.
6. Lutzner, M., Kuffer, R., Blanchet-Bardon, C. and Croissant, 0. Different papil­lomaviruaes as the causes of oral warts. Arch. Dermatol., 1982;118:393-99.
7. Clausen, F.P. Rare oral viral disorders (molluscum contagiosum, localised keratoacan­thoma, verrucae, condyloma acuminatum and focal epithclial hyperplasis). Oral Surg., 1972; 34:604-18.
8. Goodfellow, A and Calvert, H. Focal epithelial hyperplasia of the oral mucosa. A case report from the United Kingdom. Br.J. Dermatol., 1979;101:341-44.
9. Henke, R.P., Milde-Langoach, K., Loning. T. and Koppang, H.S. Human papillomavirus type 13 and focal epithelial hyperplasis of the oral mucoss: DNA hybridization on paraffin-embedded specimens. Virchows Arch (A), 1987;41 1:193-98.
10. Beaudenon, S., Praetoriua, F., Kremsdorf, D., Lutzner, M., Worsae, N., Pehau-Ar­naudet, 0. and Orrh, 0. A new type of human papillomavirua associated with oral focal epithelial hyperplasia, 3. Invest Dermatol., 1987; 88:130-35.
11. Syrjanen, SM., Syrjanen, KJ., Happonen, R.P. and Lamberg, MA In situ DNA hybridization analysis of human papillomavirus (HPV) sequences in benign oral mucoaal lesions. Arch. Dermatol. Rca., 1987; 279:543-49.
12. Clausen, F.?. Histoparhology of focal epithelial hyperplaaia. Evidence of viral infection. Tandlaegebladet, 1969; 73:1013-21.

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