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Cheilitis glandularis: a pediatric case report R ebecca Y acob i, BSc, M Sc, D D S D avid A. B row n, D D S
C heilitis glandularis is a rare disorder, usually affecting the low er lip of adults. T h is case in v o lv e d b o th lip s o f an adolescent m ale. A fa m ilia l h istory o f the con dition m ay have con tributed to the in volvem en t of both lips, an d m ay reinforce the im portan ce o f hereditary tendency in the developm ent o f cheilitis glandularis. E m otional disturbance and p o o r o ra l h yg ie n e a lso h a d a ro le in th e d iso r d e r in th is case, w h ic h w as successfully treated w ith surgery.
h e ilitis g la n d u la r is is a rare disorder of unknow n cause that was first described by V olkm ann in 1870.1 T h e co n d itio n usually affects th e lo w e r lip a n d is c h a ra c te riz e d by n o d u la r enlargem ent, reduced m obility, an d eversion of the lip w ith hypertrophy of the labial m ucous glands and dilation of the excretory ducts.2'3 It may also be associated w ith oozing of m ucopurulent d isch arg e from the p a p u le s on the lip surface.4 Progressive stages of bacterial involvem ent and in fla m m atio n (sim ple type, su p erficial su p p u rativ e type, and deep su p purative type) may be observed w ith sy m p to m s of s tin g in g a n d p a in attrib u tab le to bacteria-induced sialade nitis and m ucositis.2,5 Some of the etiologic agents that have been p ro p o sed for cheilitis g la n d u laris in c lu d e b a c te ria l in fe c tio n ,6 s y p h ilis ,1 a c tin ic d a m a g e ,5'7 e m o tio n a l d i s t u r bances,8 tobacco usage,9 an d congenital p r e d is p o s itio n .5,6 V a rio u s tre a tm e n t
C
m o d a lities have been used in the p ast in c lu d in g radiotherapy, an tib io tics, ste roids, an d surgery.4,5,10
Report of case An 11-year-old w hite m ale cam e to the dental clinic w ith a history of recurrent u lc e ra tio n a n d sw e llin g of u p p e r an d lower lips for the preceding 3 to 4 years. P ain was usually associated w ith eating. T h e p a tie n t’s m other confirm ed th at she and her brother suffered from a milder, b u t sim ila r c o n d itio n of the lip s. She also suggested th at her son was sensitive and em otional, b u t no causal relatio n sh ip could be established between the ch ild ’s em otional state an d the lip condition. E x a m in a tio n show ed b o th lip s were e n la rg e d w ith th ic k e n in g , s lig h t te n derness, and nodularity th ro u g h o u t. T h e lower lip was enlarged to a greater extent than the u p p er lip (Fig 1). Sm all, red, pin-headed lesions were evident through-
F ig 1 ■ E n largem en t a n d eversion o f b oth u pp er an d low er lip s
o u t the lip, w hich exuded a clear, sticky, m ucous secretion. C ru stin g of the lower lip w ith ulcerated areas on the lips and the left buccal region was indicative of th e s u p e rf ic ia l s u p p u r a tiv e ty p e of c h e ilitis g la n d u la ris . P a ro tid an d s u b m an d ib u lar glands were norm al. A p h o to m ic ro g ra p h of the u lcerated low er lip show ed a n in filtra te of p o l y m o r p h o n u c le a r le u k o c y te s a n d ly m p h o c y te s. A lso p r e s e n t w as a m in o r s a liv a ry g la n d s u r ro u n d e d by d ila te d fibroid ducts an d a ch ro n ic inflam m atory in f ilt r a te c o n s is tin g o f ly m p h o c y te s, p la sm a cells, a n d e o s in o p h ils (F ig 2). T h e histological diagnosis was cheilitis glandularis. In itia l th erap y w as con serv ativ e an d co n s iste d of 2% lid o c a in e (X y lo ca in e) a p p lied topically o n ulcers of the lips: tr ia m c in o lo n e a c e to n id e d e n ta l p a s te (Kenalog in Orabase) ap p lied to the lips th re e tim es a day; b e ta m e th a s o n e d i s o d iu m p h o s p h a te (B e tn e so l) p e lle ts dissolved in the m o u th twice a day and a m o u th rin se w ith a so lu tio n of te tra c y c lin e e lix ir 40%, d ip h e n h y d r a m in e hydrochloride (Benadryl) 40%, an d a n ti diarrhea m edicine (Kaopectate) 20% twice a day. T w o m o n th s after in itia tio n of treatm ent, the lips appeared sm aller w ith n o ev id en c e of u lc e r a tio n o r m u c o u s secretio n . T h e p a tie n t also re p o rte d a reduction in discomfort. Six m onths later, how ever, sw ellin g of th e lip s was still q u ite extensive an d the ch ild had expe rienced recurrent ulcerations of the lips. T h erefo re, th e p a tie n t w as referred to th e d e p a rtm e n t o f p la s tic su rg e ry for s u rg ic a l tre a tm e n t p e rfo rm e d VA years JADA, Vol. 118, March 1989 ■ 317
CLINICAL
REPORTS
after the in itial diagnosis. Surgery consisted of elliptical incision of b oth u p p er and low er lips using sharp dissection that removed the affected tissue. T h e la rg e h y p e r tr o p h ie d g la n d s w ere also rem oved from the u n d e rly in g lip tis su e s. C lo su re o f th e w o u n d w as p e rfo rm e d u s in g 4.0 c h ro m ic su tu res. T h e postoperative course was u nrem ark ab le a n d 2 m o n th s la te r th e co sm etic result revealed an excellent lip contour (F ig 3). T h e lesion h ad n o t recurred 9 m o n th s postoperatively.
Discussion T h e c o n d itio n of c h e ilitis g la n d u la ris is usually associated w ith m iddle or old age in m a les,2'3,9 a lth o u g h a few cases have been reported in young children5,6 and females.8 M ost incidences in the adult g ro u p have been re la te d to lo n g -te rm e x p o s u re to su n , w in d , d u s t, o r tobacco.9,11,12 C h ro n ic ac tin ic, clim atic, an d chem ical irritatio n may cause scar rin g an d eversion of the lip. As a result of the eversion, the oral m ucosa is exposed to irritatio n to w hich it is unaccustom ed, leading to the hyperplasia of the m ucous lip glands w ith thickening an d in flam m atio n of their ducts.9 In the pediatric case reported here, the edolo g ic factors a p p e a r e d to be p o o r o ra l h y g ie n e , e m o tio n a l d istu rb a n c e , a n d a fa m ilia l h is to ry . T h e h e re d ita ry m a c r o c h e ilia co n trib uted to the pro tru sio n of the lip and exposure of the norm ally protected la b ia l m u c o sa, w h ic h in c o n ju n c tio n w ith the p o o r o ral hygiene, may have led to th e in f la m m a tio n a n d d ila tio n of the ducts. T h e co n trib u tio n of em o tio n a l d istu rb a n ce has been previously rep o rted by W oodburne an d P h ilp o tt.8 T h e lack of history of ch ro n ic actinic, c lim a tic , o r c h e m ic a l ir r i ta t io n leads to the hypothesis th a t hereditary, em o tional disturbance, and poor oral hygiene h ad a role in the condition of this young p atient. F am ilial occurrence of cheilitis g la n d u laris has n o t been observed frequently. W eir and Jo h n so n 5 reported its occurrence in a b lack m an an d h is tw o ch ild ren . It w as su g g e ste d th a t th e m o d e of in h e rita n c e is a u to so m a l d o m in a n t. It is p o ssible th a t p red isp o sin g character istics, such as poor oral hygiene, super im p o sed o n h ered itary factors, lead to m anifestation of the disease. It is in ter esting to note that the case reported here is sim ilar to that reported by Weir and J o h n s o n 5 in th e ab sen c e o f th e m o re directly associated edologic factors such 318 ■ JADA, Vol. 118, March 1989
F ig 2 ■ P h o to m ic r o g r a p h o f m in o r sa liv a ry
F ig 3 ■ C lin ic a l c o n d itio n o f the lip s 2 m o n th s
g la n d in th e lo w er lip b ein g surrou nd ed by dilated
a fte r s u r g ic a l tr e a tm e n t s h o w s a n e x c e lle n t
d ucts and a ch ro n ic in fla m m a to ry in filtra te. (O rig m ag x 290).
co sm etic result,
as chem ical, clim atic, or actinic irritation. T h e controversy of w hether inflam m ation or g la n d u la r h y p e rtro p h y in itia te s the p a th o lo g ic al changes in c h e ilitis g la n d u la ris is still n o t resolved. H ow ever, in c h ild re n it a p p e a rs th a t g la n d u la r h y p ertro p h y ap p ears relatively early in the d e v e lo p m e n t o f th e d ise a se an d probably precedes secondary in fla m m a tory changes. T h e im p o rta n ce of th is c o n d itio n is reflected in the reported 18% to 35% chance of squam ous cell carcinom a.5 Most cases w ith carcinom a have been noted in the w h ite p o p u la tio n o ld e r th a n 40 years and have been associated w ith the actinic dam age seen in the older patients. Stuller a n d o th e r s 11 p r o p o s e d th a t lo w e r lip eversion increased the su scep tib ility of th e lip s to th e e tio lo g ic fa c to rs of squam ous cell carcinom a an d that chei litis g la n d u la ris is n o t a p re m a lig n a n t condition. V arious conservative treatm ent m ethods have been suggested in the past, in clu d in g an tih istam in e s8; tria m cin o lo n e6; topical steroids an d systemic an tich o lin erg ics10; antibiotics; radiation; or corticosteroids.4 T h e c o n se rv a tiv e tr e a tm e n t a p p ro a c h w as in itia te d in th is case in h o p es of m inim izing the need for surgery. As w ith m ost conservative approaches, however, long-term im provem ent was n ot evident, a lth o u g h the severity of th e co n d itio n was reduced for a sh o rt tim e. As w ith the m ajority of cases, surgical treatm ent w as p erfo rm ed w ith e x c ellen t esth etic and functional results. R ada an d others7 proposed the elim in atio n of predisposing etiologic agents as the in itial treatm ent m odality; however, they also suggested th a t a lte rin g e n v iro n m e n ta l factors in patients w ith fam ilial ch eilitis g la n d u laris rarely achieved lastin g im provem ent w ithout surgical intervention.
Summary T h e d istin g u ish in g feature of this case was th at the co n d itio n was seen in both u pper a n d low er lips. A fam ilial history should be taken as there is a hereditary tendency to develop cheilitis glandularis. -----------------------J'Ä O A ----------------------Dr. Y acobi is o n staff a t th e H o sp ita l for Sick C h ild re n , T o ro n to , C an ad a. Dr. B row n is a p o st g rad u ate stu d en t in o rth o d o n tics a t the U niversity o f W este rn O n ta r io , L o n d o n , C a n a d a . A d d ress re q u e sts fo r r e p r in t s to D r. Y a co b i, H S C , 555 U n iv e rsity A ve, T o r o n to , O n ta rio , C an a d a M 5G 1X8. 1. V o lk m a n n R von. E in ig e fa lle vo n c h e ilitis g la n d u la r is a p o s te m a to s a . A rc h P a th o l A n a t 1870;50:142-4. 2. O liv er ID , P ic k e tt AB. C h eilitis g la n d u la ris. O ral S urg O ral Med O ral P ath o l 1980;49:526-9. 3. W inchester L, Scully C, P rim e SS, Eveson JW. C h e ilitis g la n d u la ris : a case a ffe c tin g th e u p p e r lip. O ral S u rg O ral Med O ral P a th o l 1986;62:6546. 4. J o s h i H N , D ay al PK. C h e ilitis g la n d u la ris . J O ral Med 1984;39:183-5. 5. W eir TW , Jo h n so n WC. C heilitis glandularis. Arch D erm atol 1971;103:433-7. 6. D oku H C , S h k la r G, M cC arthy PL. C heilitis g la n d u la r is . O ra l S u r g O ra l M ed O ra l P a th o l 1965;20:563-71. 7. R a d a D C , K o ra n d a FC , K atz FS. C h e ilitis g lan d u laris—a disorder of ductal ectasia. J D erm atol Surg O ncol 1985;11:372-5. 8. W o o d b u rn e AR, P h ilp o tt OS. C h eilitis g la n d u laris: a m an ifestatio n of em o tio n a l d isturbance. Arch D erm atol Syph 1950;62:820-8. 9. Everett FG , H o ld er T D . C h eilitis g lan d u laris a p o ste m a to s a . O ra l S u r g O ra l M ed O ra l P a th o l 1955;8:405-13. 10. E pinette WW, H urw itz RM. A cquired cheilitis g lan d u laris sim plex. P last R econstr S urg 1973;51:3345. 11. S tuller CB, Schaberg SJ, Stokos J, Pierce GL. C h e ilitis g la n d u la r is . O ra l S u rg O ra l M ed O ra l P athol 1982;53:602-5. 12. Sw erlick RA, C ooper P H . C heilitis glandularis: a re-evaluation. J Am A cad D erm atol 1984; 10:46672.