Focal epithelial hyperplasia in a half-breed family of Colombians
Since 1956 there have been a few reports of small numbers of instances of focal epithelial hyper plasia in the literature (Table 1). Most of these have derived from various parts of South and Cen tral America,1_5;7-9-11 but individual instances have also been reported from other areas.5-8-10
Antonio Gomez, DDS Carlos Calle, DDS Graciliano Arcila, Medellin, Colombia J. J. Pindborg, DDS, Copenhagen
Seven children in a rural family had benign lesions corresponding to those described in earlier re ports of focal epithelial hyperplasia. However, soft palate lesions and a greater age span were found in this group. Knowledge of the geographic occur rence of the condition and the role of heredity, en vironment, mitotic activity, and other factors will require further studies.
Previous studies
■ C linical aspects: A ll reported instances of familial epithelial hyperplasia have been seen in children less than 18 years of age, although Menendez6 has observed the condition in Guatemalan Indians more than 40 years of age. Among the Navajo Indians there is a girl-boy ratio of 4 to 1. Archard and others1 describe the lesions as mostly multiple, soft, sessile and papular, ovoid, nodular elevations of the mucosa. The surface is finely stippled or slightly verrucal and keratotic; most often the color is like that of the adjoining mu cosa. When the mucosa is stretched, the lesions tend to disappear. Hettwer and Rodgers5 describe these lesions as having a cobblestone appearance. Their size is usually between 0.1 and 0.5 cm. Most commonly seen in the lower lip, they some times extend out on the vermilion border; the next most frequent sites are the buccal mucosa, commissures, and upper lip. The tongue, gingiva, and anterior pillars of the fauces are infrequent sites. No lesions on either the floor of the mouth or the palatal mucosa have been reported. The lesions may disappear spontaneously. 663
T a b le 1 ■ O ccu rre n ce of fo ca l e p ith e lia l h y p e rp la s ia am ong v a rio u s p o p u la tio n g ro u p s .
Race
G e og r ap hi c location
Author
Year
Es t r a d a 2
1956
Ka tio s In di a n
Choco, C o lo m bi a
Reyes7
1962
In di a n
G u at e m al a
So ne ira and Fon seca9
1964
In di a n
To ku ko , Ve n e z u e la
A r c h a r d and o t h e r s 1
1965
N a v a jo Ind ia n Eski mo U n s pe ci fi e d a b o ri g in a l or ig in Xa va n te I n d ia n
G al lu p, N.M. Alaska
W it k o p a n d N i s w a n d e r 11
H e tt w e r a nd R o d g e r s 5
1965
■Xa va nt e I n d ia n L a d in o ( m i x t u r e of Sp an is h and Pipil I n d ia n ) Q u ic h e May a In di a n
1966
No. No. wi th pe op le foc al e p i th el ia l e x a m in e d hy p e rp l a s ia 75
2
1
1
160
54 25* 1 2 1
So u th w e s t USA M a to Grosso, Brazil M a to Grosso, B ra z il Sa nta Ana , El Sal vador
206 1,127
7 2
Sa nta Ma ria Ca uq ue , Gu at e m al a
64
2
1
1
Pol yne si an
Bo rn in Ha wa ii of S a m oa n p ar en ts
Sewerin.8
1968
Ca ucasian
Copenhagen, Denmark
1
1
W a ld m a n and S h e lt o n 10
1968
Caucasian
NC, USA
1
1
Pr e s e n t st ud y
1968
H a lf -b r e e d wi th N e gr o i n fl u e n c e
A n ti o q u i a , Colo m bia
11 (o n e f a m i ly )
7
* Only 15 we re r e p o r t e d in detail.
■ Histopathology: The chief morphologic changes found by Archard and others1 consist of localized nodular elevations of the oral epithelium that exhibit acanthosis or hyperplasia of the spinous cell layer, and result in thickening, elongation, and anastomosing of the rete pegs. Minimal parakeratosis is present in most specimens. Fur thermore, a considerable mitotic activity (in some specimens) and a ductal hyperplasia are seen. Soneira and Fonseca9 also studied biopsy speci mens from the lesions. Although they termed the histologic changes leukoplakias, their photomi crographs clearly show the same changes de scribed by Archard and others. ■ Etiology: No definite etiologic background has been established for focal epithelial hyper plasia. Local factors operating in the oral cavity have been considered, but no significant com mon irritating factor has been found.1 Because of the similarity of the lesions to viral warts a viral etiology has been contemplated, but inclusion bodies have not been demonstrated. One of the patients in Archard’s study,1 and two of those re ported by Soneira and Fonseca9 had warts on the skin of the face. Finally, vitamin deficiencies have been suggested as a possible factor.9 664 ■ JADA, Vol. 79, Septem ber 1969
Report of case Inasmuch as a familial occurrence of focal epi thelial hyperplasia has not yet been described among Colombians, and because the findings dif fered somewhat from those of Archard and others1 and Witkop and Niswander,11 it is considered of interest to report on a family living in Medellin, Colombia. The first instance of focal epithelial hyperplasia examined in the dental school of Medellin was found in a 17-year-old girl, (case 6 in Table 2). She mentioned that other members of the family (Fig 1) were similarly affected. A ll members of the family were subsequently examined. The family, comprising father, mother, and nine children, were typical members of a rural group in Pueblo Viejo, County of la Estrella, a short distance outside of Medellin, Antioquia. Their physical appearance suggested half-breed Indian with a possible Negro influence. ■ C linical findings: The father (52 years old), the mother (49 years old) and two brothers (21 and 22 years old) were not affected by focal epi thelial hyperplasia. Table 2 summarizes the clin ical findings for the seven affected members of
Fig 1 ■ Seven members of family group of 11 examined in this study.
the family. The lower labial mucosa was affected in all of the patients and the buccal mucosa (Fig 2) and borders of the tongue (Fig 3) in six pa tients. The next most frequent sites were the soft palate and the upper labial mucosa. The lesions were often multiple in one location; in one of the patients, not less than 17 lesions were found on the lower labial mucosa. The lesions were uniform in appearance in the various children although the size and number varied. In a few locations, the lesions consisted of small, pedunculated ex crescences. Most of them, however, were sessile, flat, large (up to 3.0 X 2.0 cm), slightly reddish, papular, elevations of the oral mucosa. Some had a cauliflower appearance or a whitish hue.
right). The surface layer was slightly parakeratotic, but in some places large, vacuolated cells were ob served in the superficial layers. These cells con tained slight amounts of glycogen. There was a narrow zone close to the surface showing keratohyaline granules. There was increased mitotic activity with mitoses occurring in the spinous cell layer in the epithelium and, in the first two speci mens, a number of cells with enlarged nuclei and even some multinucleated cells. The mitotic ac-
■ Histologic findings: Four biopsy specimens were taken from Case 6. The first and second were taken from the buccal mucosa when the patient was 14. When she was 17, two more biopsy speci mens were taken, one from a lesion in the buccal mucosa and one from a lesion on the mucosal area of the lower lip. The histologic features were quite similar in all four. The epithelium was slightly hyperplastic with acanthosis (Fig 4, left) and elongated, anastomosing rete pegs (Fig 4, Table 2 ■ Clinical findings in focal epithelial hyper plasia in seven children of one family. Site o f le sion s Case no.
C h ild’s ag e & sex
Labial mu cosa
Buc cal
Tongue
U p p e r Lower 1 2 3 4 5 6 7 * Tw in s
10 11 12 12 15 17 23
M F F* M* F F F
+ +
+ + + + + + +
Palate H ard
+ + + + + +
+ + + + + +
Soft
+ + + +
Fig 2 ■ Case 6. Focal epithelial hyperplasia affecting buccal mucosa. Gomez—others: FOCAL EPITHELIAL HYPERPLASIA ■ 665
tivity seemed somewhat greater in the first two specimens.
Discussion Clinically as well as histopathologically there seems to be no doubt that the seven children in the family described in this report suffer from the same condition described by Estrada,2-4 Reyes,7 Soneira and Fonseca,9 Archard and others,1 and W itkop and Niswander.11 Some differences were
Fig 3 ■ Case 4. Focal epithelial hyperplasia affecting tongue.
found, however, when a comparison with the previously reported cases was made. It is the first time that focal epithelial hyper plasia has been described among a half-breed group with Negro influence. Lesions of the soft palate have not been seen by previous investi gators. W ith regard to age, Archard and others1 found an age range from 3 to 13 years, and Soneira and Fonseca9 and W itkop and Niswander11 did not see focal epithelial hyperplasia in any person older than 18. Our material includes a 23-yearold woman. Archard and others1 found, in two instances, that one other child in the family was affected, and W itkop and Niswander11 observed the le sions in two first cousins, but a familial occurrence such as the one described in this paper has not been reported previously. Previous authors have speculated about a hered itary background for the lesions, but are cautious not to exclude the possibility of an infectious dis ease caused by a virus. As our findings also sug gested heredity, we decided to try to rule out the possibility of an infection occurring among the children in the surroundings of the affected fam ily or among their playmates. Eighty children were studied— 35 boys and 45 girls ranging from 2 to 13 years of age. The children comprised halfbreeds as well as whites, but the social background was difficult to establish as the population in the area is a very mixed one. None of the children showed any signs o f focal epithelial hyperplasia. The histologic study of case 6 revealed higher mitotic activity and irregularities of the nuclei, especially in the spinous cell layers in the first
Fig 4 ■ Case 6. Photomicrograph of biopsy specimen from lesion on lower labial mucosa. Note acanthosis of rete pegs (left). Photomicrograph of biopsy specimen from lesion on right buccal mu cosa shown in Figure 2. Note anastomosing rete pegs and presence of vacuolated cells in surface layers (right). 666 ■ JADA, Vol. 79, Septem ber 1969
biopsy specimens when they were compared with the second batch three years later. It could be as sumed that a decrease in mitotic activity causes the lesions to disappear in adults. Futher studies are necessary to prove this hypothesis. Spontane ous regression has been observed by Archard and others,1 but extensive long-term studies should be carried out to clarify fully the clinical behavior of this condition. Hettwer and Rodgers’5 observation of focal epi thelial hyperplasia in a Polynesian girl is extreme ly interesting in the light of the migration of people between the Americas and the islands of the Pacific. The reports of unquestionable focal epithelial hyperplasia in a Danish girl8 and in an American woman11 naturally raise the question whether the condition is also to be found among whites. Epidemiologic studies are very desirable in order to map out the geographic occurrence of focal epithelial hyperplasia.
Summary Focal epithelial hyperplasia is a benign lesion characterized mostly by multiple, flat, verruca like elevations of the oral mucosa. So far, it has been described in Indians from Brazil, Colombia, Guatemala, and New Mexico, and in Ladinos in El Salvador. This paper reports on the occur rence of focal epithelial hyperplasia among seven out of nine children in a Colombian half-breed family of Indians with Negro influence. The his
tologic findings consist of epithelial hyperplasia with minor nuclear variations. A possible heredi tary racial background is discussed.
Doctor Gomez is auxiliary professor, dental school; Doctor Calle is chief of the oral medicine department and professor in the dental clinic; Doctor Arcila is chief of the depart ment of anthropology, University of Ar\tioquia, Medellin, Colombia. Doctor Pindborg is professor of oral pathology, The Royal Dental College, 4 Universitetsparken, 2100 Co penhagen 0 , Denmark. Requests for reprints should be ad dressed to Doctor Pindborg.
1. Archard, H.O.; Heck, J.W.; and Stanley, H.R. Focal epithelial hyperplasia: an unusual oral mucosal lesion found in Indian children. Oral Surg 20:201 Aug 1965. 2. Estrada, L. Aporte al estudio odontologico de los Indios Katios. Heraldo Dental 2:5, No. 7, 1956. 3. Estrada, L. Informe preliminar sobre algunos aspectos odontologicos de los Indios Caramanta. Bol Inst Antropol Univ Antioquia 1:319 1956. 4. Estrada, L. Estudio medico y odontologico de los Indios Katios del Choco. Temas Odontologicos 7:198 1960. 5. Hettwer’, K.J., and Rodgers, M.S. Focal epithelial hy perplasia (Heck’s disease) in a Polynesian. Oral Surg 22:466 Oct 1966. 6. Menendez, G.R. Personal communication. 7. Reyes, D.G. Verruga de la cavidad oral. Riv Colegie M e d (Guatemala) 13:223 1962. 8. Sewerin, I. Fokal epitelial hyperplasi. Et tilfaelde. Tandlaegebladet 72:610 Aug 1968. 9. Soneira, A., and Fonseca, N. Sobre una lesion de la mucosa oral en los niños Indios de la Misión los Angeles del Tokuko. (Comunicación previa). Venezuela Odont 29:109 1964. 10. Waldman, G.H., and Shelton, D.W. Focal epithelial hyperplasia (Heck's disease) in an adult Caucasian. Oral Surg 26:124 July 1968. 11. Witkop, C.J., and Niswander, J.D. Focal epithelial hyperplasia in Central and South American Indians and Ladinos. Oral Surg 20:213 Aug 1965.
Gomez—others: FOCAL EPITHELIAL HYPERPLASIA ■ 667