ORAL
PATHOLOGY
American Academy of Ora Pathology Dona.ld
l
Kerr,
Editor
**.*.*.*
ORAL LUPUS
l
MANIFESTATIONS
..*
OF CHRONIC
l
.,........
DISCOID
ERYTHEMATOSUS
Report
of a Case
National
Institute
of
Llenttrl
Rrscrrrdi
is a c~llagc~r disease which o~urs in a chronic discoid form and in an acutc form. In chronic discoid lupus, the lesions are usually present on the face, where thoy inroluc thtz checks and itritlge OT the nose and follow a butterfly tlistr4bution. liesions may also 1)~ seen on the scalp, Cars, neck, and cxtrcmititls, where tllcy OCCLII’ usuull~ as c~rythcmatous, sealing plaques with focal atrophy and follicular plugging. The acute form 01’ the disease may tlc~clop indcqx~ntlrntl; OP from a ~PC’existing discoid form. (‘utancous marriftistatiorrs may he widely spread over the body, occurring usually as erythcmatous. etlemat,ous maculrs with patchy ant1 coalescent distribution. T’csicles, hallac, or papulcs rnay hc seen. Systemic s\mptoms arc prcscarit and include any oi a number of signs and symptoms, such as fever, arthralgias, myalgias, anorexia, malaise, Icukoponia, thrornhocvtopcnia, splcnomegaly, pamma glolmlincmia, \ill\Tulal diseases, diarrhea, and dpsphagia. In hot,h forms, mucous membrane involvcrnent may hc SPW.
L
T~PUS ERYTHEMATOSTJS
t:r;trwll. Nativn:il 1nstitutr llf ikwtal Iiesrarch. *Clinical Associate Clinical Invcstigatiom National Institutes of Health. **Formerly Senior Assistant Dental Surgeon. United States Public Health Service Hospital, Federal Correctional Institution, Seagoville, Texas. ***Chief. Oral Medicine and Surgery Branch, National Institute of Dental Research. National Institutes of Health.
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in the literature; even fewer of these include discoid lupus erythernatosus histologic descriptions of oral mucosal lesions. The early literature is of little value, since no distinction was made between different types of lupns erythematosus. Howe~cr, some rcport,s did indicate rather frequent involvcmcnt of the muco~~s membrancs.“~ I8 Most studies of systemic lnpus crythernatosus haye reported oral mucosal involvement, ranging from 15 of 138 case? to 18 of 41 cases.:’ More recently, in 1961, Marten and Blackburnl’ rcl)ortcd on 51 cases of chronic discoid lupus, among which they found 3 cases with oral mucosal manifestations. dlso in 1961, a case report by Feinstein7 documented the histologic characteristics of the oral lesion seen in chronic discoid lupus erythemntosus. Shklar and McCarthy13 and Shklar and MeyerI have reported in greatest detail the microscopic findings in the oral lesions of chronic discoid Inpus crythcmatosus.
CASE REPORT A 28.year-old lvhite man of Mexican descent had skin lesions of the face and ears which had developed over a period of 9 months. These started as small reddened areas which became raised, thickened, and occasionally itched. One week before admission to the hospital the patient began having some achiug pain in the right knee, and this was followed in several days by a similar aching in the left knee. These symptoms had occurred interA l&gear-old sister of the patient had died 2 mittently up to the time of examination. years earlier with a 4 year history of systemic lupus erythematosus. Another sibling developed nephritis following scarlet fever. Four other siblings and both parents were living and well. The patient denied having any other symptoms or a history of fever and chills, although he claimed to be sensitive to sun exposure anI1 occasionally had headaches related to a burning sensation of the eyes. Physicnl ~za?nination.-Temprrature, pulse, blood pressure, and respiration were within normal limits. There was a 1.2 Ly 0.8 em. skin lesion about the left posterior auricular region; this was flat and erythematous with a shiny, atrophic surface. An erythematous
Fig.
l.-Clinical
lesions
of discoid lupus on thr left atrophir areas \vitb a .slightIy
car appeared as erythematous, scaly appearance.
flattened,
Fig. L-Histologic specimen of the postcriw auricular lesion reveals a circumscribed atrophic area of skin which displays hyperkeratosis. liquefaction degeneration, and follicular plugging. A patchy infiltrate of lymphocytes is seen in the dermis. (Hematoxylin and eOsin stain. Magnification, X14 ; reduced ‘/.)
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first and second molar area (Fig. 2). A similar 0.7 by 1.3 cm. lesion was noted on the buccal alveolar mucosa of the upper left first and second premolar area (Fig. 3). These lesions appeared reddened, atrophic, indefinitely outlined, and slightly elevated at the margins. Numerous small dilated blood vessels were seen at the margins, and the centers of the lesions revealed early scarring with a diminution of their inflammatory character. Laboratory Data: The chest roentgenograrn, elect.roeardiogram, urinalysis, complete blood count, hemoglobin determination, and red blood count were all normal. The white blood cells numbered 7,100, with 26 per cent neutrophils, 6.5 per cent I!-mphocytes, 25 per cent atypical lymphocytes, 1 per cent juvenile, 6 per cent bands, and 2 per cent eosinophils. The sedimentation rate was 5 mm., and a repeat sedimentation rate was 13 mm. On two occasions the lupus erythematosus preparation was negative. Total serum protein was 2.83; blood urea nitrogen, 14.5 mg. per cent; 5.57 Gm. per cent; albumin, 2.74; globulin, total cholesterol, 252; alka1ir.e phosphatase, 3.5 Bodansky units; thymol turbidity, 3.9 1-t at 24 hours and 2+ at 48 hours; total serum MacLangen units; cephalin flocculation, 47 per cent; phenolbilirubin, 1.15 mg., direct 0.65 mg., indirect 0.5 mg.; hematocrit, sulfonphthalein excretion, normal. Repeat Kahn and Kolmer tests were nonreactive. Histologic Findings.-Representative biopsy specimens were taken from the posterior auricular lesion (Fig. 4), the lesion on the left face, and the lesion from the attached buccal gingiva of the upper left premolar region (Figs. 5 and 6). Posterior azll-ic&r &ion (Fig. 4) : Sections revealed a circumscribed area of chronic inflammation and atrophy of the skin. The epidermis had a shallow discoid depression, with hyperkeratosis and keratin plugging of the thermal appendages, marked atrophy of
Fig. 5.-Histologic specimen of lesion from the left alveolar mucosa ve als a uniformly atrophic epithelium and dense lymphocytic infiltrate an Id eosin stain. Magnification, x55 ; reduced $&..)
(seen below.
in Fig. 3) re( Hematox ylin
E’ig. 6:-Histologic detail of gingival epithelium seen in Fig. 3 in xvhictr liqurfadion (lctq%oration. the atrophic nature of the cpithelium, and the lyrnphocytic infiltrate ;tr~ r,vidont. (Periodic acid-Schiff reaction. Mapnificntion. X500 ; reducwl $A.)
IWWUYYION )_ L
Of the three casrs of vhronjc tliscGt1 lupns cq+henlatosus wit,h rnucosal involvement clescribctl by Martc~n and P,laclrbur~~,‘~ two had “typical red patches with slightly deprcssrtl white vlantvs” p~sc~nt OII the buceal mucosa. In the third patient white pat,c*h(ssalotlv WCIT p~~~~ni, This last patient had suffered from rccurrcnt ntouth ulcers l’or III:II~J’ ycv~t’s, ant1 the white patches lesions 1~~1 IKY~ prcscnt in these may have been an end result of th(lsv. ‘1’1~~~ three patients for 5 years. In that series of fifty-one cases none of the other
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cases of chronic discoid or generalized discoid lupus erythematosus showed any mucosal involvement, but in one of the two cases of subacute disseminated lupus erythematosus there were buccal lesions. No histologic description was given. Feinstein7 reported a case of chronic discoid lupus with a 31 year history. He described oral lesions near the commissure and in the floor of the mouth. A biopsy of the latter area revealed “a rather wide zone of chronic inflammatory cell infiltration” and pseudoepitheliomatous hyperplasia. Shklar and co-workers,13, l4 in two articles, discussed the histologic characteristics of the oral lesions of chronic discoid lupus erythematosus. The classic picture of the oral lesions, according to Shklar and Meyer,14 “consists of a modcratc parakeratosis or hyperkeratosis, hydropic degeneration of the stratum germinativum, degcncration of the connective tissue collagen, and an infiltration of chronic inflammatory cells,” primarily lymphocytic, tending to be arranged in a perivascular pattern. They add, however, that though the infiltrate is invariably distributed perivascularly, there may be an extremely dense infiltrate and, therefore, the perivascular arrangement may be obscured. Nevertheless, collagen degeneration is a “constant feature” and they find that periodic acid-Schiff reaction is particularly useful in delineating this. The histologic features of the oral lesions of chronic discoid lupus erythematosus appear at present to be suggestive of, but not specific for, such a diagnosis. It is to be hoped tha,t, with further documentation of cases ‘of chronic discoid lupus exhibiting lesions of the oral mucosa, characteristic histomorphologic features may emerge. The uniform band of chronic inflammatory cells seen in the present cast may represent a different stage in t.he course of the oral lesions, or, as Shklar and Meye? point out, it may reflect secondary involvement of the gingival tissues by contiguous inflammatory processes. The thinned atrophic epithclium may be similarly explained. However, the hydropic (or liquefaction) degeneration of the stratum germinativum, the moderate parakeratosis and hyperkcratosis, the thickening of the basement membrane and vessel walls, and the lymphocytic infiltrate are findings in this case that are comparable to those described by Shklar ancl MeyerI’ for the oral manifestation of chronic discoid lupus erythematosus. The differential diagnosis of t,his oral lesion must include lichen planus and pemphigus primarily, as well as lymphorytic infiltrates, such as lymphocytic lymphoma. Neither lichen planus nor chronic discoid lupus erythematosus in its oral manifestations can be diagnosed solely on the basis of t,he histopathologic appearance. Cobb require clinical correlative findings to support the diagnosis. It is thought that the clinical appearance of the oral lesions, the cutaneous lesions, the history, and the laboratory findings fully support such a diagnosis in the case reported here. CONCLUSION A case of chronic discoid lupus erythcmatosus is reported, and the oral and cutaneous manifestations are clinically and histologically described.
REFEREECCES
1. Allen, A. C.: The Skin, St.. I,ouis, 1954, l’hc C. V. Mosby (lompany. 2. Anderson, W. A. I).: Pathology, etl. 4, St. Louis, 1961, The C. V. Mosby t’on~pan:-. 3. Bernier, J. I~.: The Management of Oral Ijisease. St. T,ollis. 1955, The (‘. V. >Iosl~v . Company. 4. Rhaskar, 8. N.: Synopsis of Oral Pathology, St. Louis, 19ti I, The C. V. Mnsby Company. 5. Burket, L. W.: Oral Medicine, ed. 3, Philatlelphia, 1957, .J. H. Lippincott Company. 6. Culver, G. I>.: Lupus Erythematosus of the Muc~ous Memllrane; Report of I I Cases, J. A. M. A. 65: 773-778, 1915. 7. Feinstein, M.: Chronic IXscoi(l Lupus F;r!,thenl:ttosus, J. (‘Iin. Stomatol. (lonf. 2: 16-18, 1961. E.: Hystemic I,upus 8. Harvey, A., Shulman, L., Tumulty, P.,. !:onley, C., and Hchoenrieh, E:rvthematosus: Review of the I,rtcrature ant1 (‘liniral Analvsis of 138 (:ases, Medicine 33: 291-437, 1954. 9. Jessar, R., Lamont-Havers, IV., and Ragan, C.: Natural History of I,upus Ervt~hematosun Disseminatus, Ann. Tnt. Med. 38: 717, 1953. 10. Lever, W. F.: Histopathology of the Skin, Philadelphia, 1961, .I. B. Lippincott Company. A F’ive-year Follow-up of 77 11. Marten, R., and Blackburn, R.: Lupus Erythematosus; Cases, A. M. A. Arch. 1)ermat. 83: 430, 1961. 12. Shafer, W., Hine, M., and I,ev,v, R.: A Textbook of Oral Pathology, Philadelphia, 1958, W. B. Saunders Comnanv. 13. Shklar, G., and McCarthy: P: L.: The Oral Lesions of I,ichen Planus,, ORAI. s,T'RU., ORAL MED.& ORALPATH. 14: 164,196l. and His&hem&try of 1)ermatologic 14. Shklar, G., and Meyer, I.: The Histopathology Lesions in the Mouth, 0aa1, SURG., ORAL MED. 8: ORAI, PATH. 14: 1069, 1961. 15. Smith, T.: Mucous Membrane I,esions in Lupus Erythematosus, Rrit. .I. 1)ermat. 18: 59, 1906.