Hereditary gingival fibromatosis

ORAL

PATHOLOGY

General Section .

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HEREDITARY Report

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GINGIVAL

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FIBROMATOSIS

of a Family

s. Jerome Zackin, Boston,

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D.X.D..*

rrd DcltGd Weisbwrger, D.M.D., N.D.,“”

Mass.

gingival fiblomatosis is a KIN oral ttiscasc with a wide variet,.v of names.’ ’ ‘ Hcrcditnry or idiopathic hypcrplasia of the gums, ’ ’ “ elephant,iasis gingivac, ’ ’ ‘ ’ fibromatosis gingivae, ” “ symmetrical fihroma of the palate,” and “ congenital macrogingivae’ ’ are but a few of the t crms applied to this condition. During the past 100 years, there have been published reports of approximately 100 cases in which the definitive diagnosis has been established.z In the characterist,ic clinical picture firm, enlarged gingival tissue covers most of the anatomic crowns of I he teet,h. In most cases, the teeth are fully erupted in relation to the alvcolnr bone. The tissucl is of normal color, stippled, nonpainful! and free from hemorrhage. Usually the hyperplasia is first noted at about the time of eruption of the permanent incisors, although Hine” mentions a number of cases in which the Ruggles+ and Raynal” condition was evident during thta deciduous dentition. roportcd cases in which the defect appeared to be present at birth. Histologically, the epithelium displays a moderate hyperplasia with hyptrkeratosis and elongation of the rclte pegs, but the illcreased tissue mass is priiu;lt*ily tlltl result ol’ a lllilYl<(‘tl irlcrcasc ;1.11(11hickening ol’ tllch c~ollagc~nou.; bundles in the connect,ive tissue stroma. This tissue shows a high degree of differentiation, few young fibroblasts being present. Although the presence of chronic inflammatory cells is generally conceded to bc only an incidental finding, the amount of inflammation depends upon the arca of tissue st.udied. There is marked infiltration of chronic inflammatory cells in the region of the gingival sulcus and close to the surface, whereas the remainder of the tissue is relatively EREDITWKY

**Professor of Dental Medicine, Chief of Dental Service, Massachusetts

Harvard School of Dental General Hospital. 828

Medicine;

Oral

Surgeon

%n*l

free of inflammatory elements.“, 7 ,\pparent bony involvement, in the form of osteofibrosis, has been reported only once.’ There is strong evidnnce that many CRSCSof gingival fibromatosis occur 109 casts, fifty-four paGents were the on a hercilitary basis. 1n Rushton’s of iuarriagcs between affected and normal persons. Nevertheless, OffSplGlg sporadic C~SCSar(’ not 1Incommon, and most, case reports make no mention of affect.cd rclativcx The nunllw of pedigrees reported is not large. Perhaps the most c3tnplctc~ly docutncntccl gc~~~log~~ was that tq~orlcd by \\T(+i,” co\cring five generations and nine marriages between norm;11 and affected persons. Of the thirty-eight offspring of these marriages, nine females and six males were affected. Mirolli” rtq~ortrd on four generations in which there were twentyOf thcsc offspring. seven females and four eight offspring ho111 ten niarriagcs. malts were affected to various degrees (and some of thescb were doubtful). Savara and associalcs” rq)ortcd a family in which the father and six of eight children had the tlei’clc+. In all of these pedigrees, iIs well as in other less complete records, inheritancc~ was only tlirol@ an affected parent. This would indicate thn.t gingiral fibromatosis is undoubtedly transmitted 1))~a dominant, autosomal gone. On the 011wr Iliand, Ccrnea and co-workers” rciportcd a cast in which ai1 unaffc~ctctl woman t ransmitlcd the defect. Ituggles” described a family in which thorcl wcrc’ ten casts oC gingival fibromat osis in three generations, hut hc CliliInctl that ml>- the affected female n~cmhws transmitted the defect. A nmnlm ol’ OIher ahnorrnalitics 11are been xq~orted in association with hweditarv yitlgivnl fihromatosis, althongh only Il\-prrkichosis has been noted 1tlOR thall it ft?\\’ tilllCS.” “-” It. should be noted, however, that the incidence of the two al)normalities occurring together is small as compared with Ihe total nntnl)cr of CilSC!S of the pingival defect. .1lthonph it has l~cn stated that the treatmcwt of choice is surgical removal of the c~nlargcd tiss1ws,“, : there is a tendency for the caondition to recur within a fC\\’ T.(l;~l’s,“.‘(i, 17 A total of about fourteen hours of radium therapy to each jaw prior IO gingi\-al surgery did not seem particaularly effective in preventing rccurrtnce.13 On the other hand, extraction of the teeth causes the enlarged gitlgi\Yll tissue to shrink almost, to nornml.l” ~Ilthol~gll Il(~ath”’ illld Kcx~p~orgis”’ advocated removal of the underlying alveolus along with the teeth, tooth extraction alone is generally considered sufficient to prevent recurrence. CASF L > REPORT X family of ltalian ancestry has been followed at the Harvard School of Dental Nedieine alld the Massachusetts General Ilospital over a twenty-two year pcriocl. Fig. 1 shows the g~~rwalogy of this famil~r ; six marriages I,rt\vcvql :dfwl(vl alld 11trrn1d p,“s”ns O\,‘I’ fOllI~ generations have produced c4cveu aEectcd childreil and ten normal children. Transmission was only by an affected parent. The great-grandmother probably had the defect, but she KRS edentulous when the family first came under the cart of the dental clinics ancl therefore :I drfinitivc~ tliagnosis 1-0~1~1 not Iw nratl~. Eswpt for on,’ “-yWrWlCl IBOY,” all thrJ normal children have most or all of their permanent dentitions. In this family the condition was first noted when the children were about 21/, years of age. There is a slight hypertrichosis iu all members of the family. All other physical findings are within normal limits.

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The teeth and gingival tissues of a brother aud sister art’ shown in Figs. 2, 3, and 4. Less than one-third of the anatomic CI’OWIM of the teeth arc sreu protruding through the tile teeth IWP~ seen to be fully erupted gingival tissues, even t,hough at thcl time of opwation

Fig. 2.

Fig-.

3.

Fig. 4.

Fig. f.-Hereditary gingival Abromatosis in a l;-year-old girl. Note the greatly thickened gingiva and the maloositioning of the teeth. Fig. 3.-Hereditary gin&al fibromatosis in the 16-year-old brother of the girl shown in Fig. 2. Note the stippled, nodular appearance of the gingival tissue. Fig. 4.-Maxillary arch of the boy shown in Fig. 3. One-third or less of the anatomic crown is exposed to the oral cavity. In some areas, the tissue is smooth because of contact with the lower gingival tissue during chewing.

with respect to the alveolar bone. 1-n some areas, there xyas contact between the gingival tissues of the opposing jaws when the teeth were occluded. Because of the enlarged tissue, the muscles of the face were tense and drawn and the lips were everted.

(Alagnificntioll. n3i ; reduceil ‘4. J B.-Normal gingiva. Increase11 thickness of the connective tiss B.-Hereditary gingival fibromatosis. X 35: reduced %. primarily responsible for’ the clinical kt~~>t:arance. (Magnification,

Fig. 7 .--Same section epithelial hyperplasia with and moderate hyperkeratosis. ~1llCCd l/1. )

as shown in Fig. 6. Note the elongation of the rete pegs (Magnification, Xl00 : re-

Fig. R.--Same section as shown in Figs. 6 and 7. Note the interlacing bards of dense collagen and the large nodular zones of chrorlic inflammatory cell infiltration. (Magnification. Xl00 : r&:r;,-==~! I/- )

~~un~lx~rs of lymphocytes and rnrr plasma cells atltl ncutrophilic~ poly~r~orphonuclear leukocytc,r. There is downward proliferation of the rete lqs, both as sharp needle points and as broad, There is moderate kcratinizaflat sheets. Alternating with these are short. areas of xtroph?. tion of the epithelium.”

Fig.

9.-Alveolar

after

ridges

of the

extraction

of

mother of the all the twth

children shown in Figs. 2, 3, and 4 cxight an11 rcmovnl of the excess tismc.

years

The mother of the two children pictured in Figs. 2, 3, and 4 underwent gingivectomy of the upper anterior region at the Massachusetts (icnoral Bospital in 1938. Less than a year The tissues had reafter surgery, she noticed thxt the gingivac ~1ere slowl,v enlarging. gressed to their former condition when, iu 1930, she dcxide~d to have all her teeth extracted Accordingly, the teeth ~VCI’C rcllloved, the excess gingirae were and full dentures inserted. resected, and full dentures mere constructed in 1951. Since that time, the patient has woru the dentures on normal ridges \vithout inrident (Fig. 9).

Fig.

lO.-Hereditary

plete tures. teeth, years, mouth

Because of this experience, her two children and oue brother also underwent cornfull denextractions, with removal of the redundant tissue, and are now wearing One boy, a cousin of the children shown in Figs. 2, 3, and 4, preferred to retain his so the enlarged gingival tissues were removed by electrocautery. In the ensuing two Figs. 10 and 11 show this boy’s there has been a moderate regrowth of tissue. before and two years after removal of affected gingival tissues by electrocautery.

*Pathology chusetts General

gingival

report Hospital.

by

fibromntosis in a 13-year-old shown in Figs. 2, 3, and

Sanford

Irwin

Roth,

M.D.,

4.

Clinical

boy,

the

Fellow

cousin

in

Of the

Pathology.

Children

Massa-

Volume 14 N,,mber : SUMMARY

The genealogy of z family \\ith heredital+J gingival fibromatosis has hccfl presented. Six marriages between normal and affected persons over four generations resulted in eleven affected children (six girls and five boys) and ten normal children. The defect was transmitted nnly t,hrongh an affected

.I!%. ll.~Hew(litary

gingival flbromatosis. Regrowth of tissue two years by electrocautrry. (Same boy as shown in Fip. 10.)

follo\ving

removal

parent, the mechanism undoubtedly being a dominant autosomal gene. The clinical and histologic pictures have been illustrated, anal the treatment has been described. The literature on hereditary gingival fibromatosis has been briefly reviewed. REFERENCES 1. Kerr, 2. 3. 4. 3.

D. A.: Classification and Terminology for 1951 Periodontal Workshop, J. Am. Ih?nt. A. 44: 621-624, 7952. Rushton, M.A.: Hereditary or Idiopathic Hyperplasia of the Gums, D. Practitioner & D. Record 7: 136-146, 1957. Hine, M. K.: Fibrous Hyperplasia of the Gingivia, J. rim. Dent. A. 44: 681-691, 1952. Ruggles, S. D.: Primary Hypertrophy of the Gums, J. A. M. A. 84: 20-24, 1920. Raynal, T.: Deux cas d’hypertrophic gingivalc, gcweralisw c~ongi~nital~~ clt t’:!milial~~,

Marseille m&l. 46: 577-590,1909.

6. Ball, E. I.: Case of Gingivoma, or Elcphantiasis of the Gingira, J. Periodont. 12: 96-100, 1941. 7. Orban, B.: Discussion of Ball, E. I.: “Case of Gingivoma, or Elephantiasis of the Gingiva,” J. Periodont. 12: 100-103, 1941. 8. Englert, R. J., and Leuin, I. S.: Diffuse Osteofibromatosis; a Symptom Complex, ORX, SURG., ORAL MED. & ORAL PATH. 7: 837-841, 1954. 9. Weski, H.: Elephantiasis Gingivae Hereditaria, Deutsch. Monatschr. Zahnh. 38: 557-584, 1920. 10. Mirolli, A.: La elephantiasi familiare delle gengive, Arch. ital. di chir. 29: 401-416, 1931. Gingival Fibrosis 11. Savara, B. S., Suher, T., Everett, F. G., and Burns, A. G.: Hereditary -Study of a Family, J. Periodont. 25: 12-21, 1954. 12. Cernea, P., Garlopeau, F., Marie, M., and Sand, M.: Lcs hyperplasies gingivales familiales, Rev. stomatol. 56: 620-626, 1955. 13. Ziskin, D. E., and Zegarelli, E.: Idiopathic Fibromatosis of the Gingivae, Ann. Dent. 2: 50-55, 1943. 14. Byars, L. T., and Sarnat, B. G.: Congenital Macrogingivae (Fibromatous Gingivae) and Hypertrichosis, Am. J. Orthodontics & Oral Surg. 31: 48.51, 1945. 15. Thoma, E. H.: Oral Pathology, ed. 4, St. Louis, 1954, The C. V. Mosby Company.

Two Cases of Hpprrtrophied Gunls anti .\lvroliz 16. Heath, C.: ‘l-r. otl~~rll. SW. Gl-lYii Tbitain 11: 18-24, 1878. Ii. &app, R., Nikiforuk, G., Donohuc, D. .W., and Williams, C. I-1. M. : Itliopathic .Hyp~r plasia of Gingivae Associated 1Vith Macrocheilia and Ankyloglossia : A Case Report, J. Periodont. 26: 51-55. 1955. 1% Bwhner, H. J.: DifWc FiGroma of the Gums: Report of ‘I’cvo (‘aser. .J. L1rn. Ilent. d. 24: 2003-2007, J 937. 19. N why, c. 1~. : A Report on IL CRSP oi” Hyperlrophicd Gum Tissues. .T. C:rnnd. Ihf. .\. 6: 1X3-186. 1940. 20. licrageorgis, B: I’.: fili?phantiasis ties gincircs, Rev. tic chir., J.‘aris 68: ::f)X~WO, I!1 1!1 ~,al~stractetl in Tntrrnat. Ahsty. Surg. 90: 461-462, 1950).