J Oral Pathol Med (2007) 36: 524–7 ª 2007 The Authors. Journal compilation ª Blackwell Munksgaard Æ All rights reserved doi: 10.1111/j.1600-0714.2007.00569.x www.blackwellmunksgaard.com/jopm Oral manifestations of systemic and cutaneous lupus erythematosus in a Venezuelan population Jeaneth Lo´pez-Labady1, Mariana Villarroel-Dorrego2, Nieves Gonza´lez3, Ricardo Pe´rez3, Magdalena Mata de Henning1 1Dental School; 2Oral Medicine; 3Medical School, Universidad Central de Venezuela Caracas, Venezuela BACKGROUND: The aim of this study was to charac- and ⁄ or arthritis to renal failure or intense nervous, terize oral lesions in patients with systemic and cutane- cardiac and haematological disturbances (1). ous lupus erythematosus (LE) in a Venezuelan group. The basic manifestations of LE occur in the connect- METHODS: Ninety patients with LE were studied. Oral ive tissue and blood vessels, but depending on the biopsies were taken from patients who showed oral mu- anatomical location and course of the disease, LE has cosal involvement. Tissue samples were investigated with been classified as systemic LE (SLE) or cutaneous LE histology and direct immunofluorescence techniques for (CLE). Cutaneous lupus erythematosus includes variety the presence of immunoglobulins G, M, A and comple- of LE-specific skin lesions that are subdivided into three ment factor C3. categories: chronic CLE (CCLE), subacute CLE (SCLE) RESULTS: In 90 patients with LE, 10 patients showed oral and acute CLE (ACLE) based on clinical morphology lesions related to the disease. Sixteen lesions were and histopathologic examination (2–4). investigated. Oral ulcerations accompanied by white Patients with SLE frequently show cutaneous mani- irradiating striae occurred in five patients, erythema was festations during the course of the disease. Moreover, 4 observed in five patients and a white homogeneous pla- of the 11 criteria formulated by the American College of que in one patient. Fifteen lesions demonstrated vacuolar Reumathology (ACR) classification for the diagnosis of basal degeneration and 12 thickening of the basement SLE are cutaneous and oral (4). These criteria are malar membrane histologically. Direct immunofluorescence rash, discoid rash, photosensitivity and oral ulcers (5). was negative in three samples. Oral mucosal ulceration occurs in more than 40% of CONCLUSIONS: These findings corroborated that ul- patients with SLE (6–8); however, reticular, red and cers are not the only manifestation of LE in the oral white plaques have also been observed in patients with mucosa. Clinical and histological examinations are signi- SLE (6, 9, 10). The majority of these lesions showed ficant as immunoproteins are not always found on the histopathological changes specific to SLE; however, oral sample. histological and immunological patterns might be J Oral Pathol Med (2007) 36: 524–7 unspecific (11–14). The aim of this study was to describe the clinical, Keywords: lupus erythematosus; oral lesions; oral mucosa; histological and immunological manifestations of SLE systemic and cutaneous lupus erythematosus and CLE in the oral mucosa. Patients and methods Introduction Ninety patients with LE, attending the Dermatology Lupus erythematosus (LE) is a multisystem autoimmune Unit, were evaluated after filling an informed consent. disease associated with significant morbidity and mor- All patients were under immunosuppressive therapy. A tality. Lupus erythematosus occurs most commonly in careful examination of the oral cavity was performed in young women and ranges from mild cutaneous lesions each patient. Clinical recognition of oral lesions related to LE was assessed according to previous observations (6, 8, 9). Biopsies of oral lesions were taken and immediately Correspondence: Mariana Villarroel Dorrego, CC Bello Monte, Ave fixed in 10% formalin. Samples were stained with Principal de Bello Monte, Piso 3, ofc. 3 J, Caracas, Venezuela. Tel: +58 414 1716034, Fax: +58 212 7538189, E-mail: mvillarroeldorrego haematoxylin and eosin and periodic acid-Schiff (PAS) @cantv.net prior microscopic analysis. Biopsies of adjacent mucosa Accepted for publication April 15, 2007 (clinically normal appearance) were also taken and Table 1 Distributions of LE patients by gender, age, oral manifestation, histological appearance and immunopathological results LE Direct immunofluorescence Patient Sex Age classification Oral lesion Location Histopathological characteristics results 1 F 16 SLE Red macula Hard palate Hyperkeratosis, basal layer degeneration, thickening of Granular band at the basement the basement membrane zone, subepithelial lymphocytic membrane zone infiltrate and deep perivascular infiltrate, edema 2 F 29 SLE Red macula Hard palate Basal layer degeneration, deep perivascular lymphocytic Cytoid bodies Red plaque Lower lip infiltrate Ulcer surrounded by Buccal mucosa white irradiating striae 3 F 32 SLE Red plaque Upper lip Basal layer degeneration, thickening of the basement Discontinuous lineal band at the membrane zone and deep perivascular infiltrate basement membrane zone 4 F 36 CLE Red plaque Hard Palate Hyperkeratosis, basal layer degeneration, thickening Negative of the basement membrane zone, subepithelial lymphocytic Lo Characterization of oral lesions in patients with LE ´ infiltrate and deep perivascular infiltrate pez-Labady et al. 5 F 38 SLE Red macula Hard palate Hyperkeratosis, basal layer degeneration, thickening Granular band at the basement Ulcers Buccal mucosa of the basement membrane zone subepithelial lymphocytic membrane zone infiltrate and deep perivascular infiltrate, cytoid bodies 6 F 36 SLE Ulcer surrounded by Buccal mucosa Hyperkeratosis, basal layer degeneration, thickening of the Granular band at the basement white irradiating striae basement membrane zone subepithelial lymphocytic membrane zone Red plaque Lower lip infiltrate and cytoid bodies 7 F 34 SLE Red plaque Lower lip Hyperkeratosis, basal layer degeneration, thickening of the Continuous lineal band at Red macula Hard palate basement membrane zone subepithelial lymphocytic the basement membrane zone infiltrate and cytoid bodies and cytoid bodies 8 F 29 CLE Ulcer surrounded Buccal mucosa Basal layer degeneration and deep perivascular infiltrate Negative by white irradiating striae 9 F 49 CLE Red plaque Upper lip Hyperkeratosis, basal layer degeneration, subepithelial Continuous lineal band at the White plaque on Buccal mucosa lymphocytic infiltrate and deep perivascular infiltrate basement membrane zone pigmented mucosa 10 F 52 CLE Ulcer surrounded by Buccal mucosa Basal layer degeneration, thickening of the basement Negative white irradiating striae membrane zone, subepithelial lymphocytic infiltrate and deep perivascular infiltrate JOralPatholMed 525 Characterization of oral lesions in patients with LE Lo´pez-Labady et al. 526 embedded in OCT compound (Miles Scientific, USA), snap-frozen in liquid nitrogen and stored at )70°C prior to immunostaining. Direct immunofluorescence tech- nique was used in order to determine the expression of immunoglobulins (Ig) G, M, A and complement protein C3 on the tissue, using fluorescein isothiocyanate conjugated rabbit anti-human anti-sera to IgG, IgM, IgA and C3. Results The group of LE patients was composed of 8 males and 82 females with a mean age of 36.39 ± 8.42 years. Thirty-eight patients (42.22%) met the ACR criteria for Figure 2 Histopathological micrograph showing hyperkeratosis, diagnosis of SLE and 52 patients (57.78%) were basal layer degeneration and subepithelial lymphocytic infiltrate. diagnosed as CLE. All patients showed cutaneous lesions regardless of LE classification. Of the 90 patients diagnosed with LE, 10 patients observed in all cases (16 lesions) and thickening of the (11.1%), all women, showed oral mucosal lesions. Six basement membrane in 12 lesions. Lichenoid inflamma- patients were classified as SLE and four had the cutaneous tory mononuclear infiltrate was a common finding (15 form. Patient details are summarized in Table 1. None of biopsies), and although vasculitis in the deeper connect- the patient was receiving oral topical therapy. ive tissues is a common feature of LE, it was only found Oral lesions ranged from a red macula or plaque, in six cases. Periodic acid-Schiff staining did not ulcerations surrounded or not by white irradiating striae demonstrate fungal colonisation in any of the speci- to a white plaque on a pigmented mucosa (Fig. 1). mens. Clinical features varied according to the anatomical Ten biopsies of apparent normal mucosa, adjacent to location. Lesions of the hard palate were red maculae or oral lesions, were obtained. Immune deposits were plaque, in contrast, white lesions (plaque and lichen-like observed at the basement membrane zone in six biop- striae) were found only in buccal mucosa. Lesions of the sies. The pattern consisted of either a discontinuous or lips were either red plaques or ulcers. continuous lineal band or a granular band (Table 1). Microscopic analyses of 16 biopsy specimens were Positive cytoid bodies were observed in two samples. performed (Table 1) (Fig. 2). All cases showed epithelial Three biopsies were completely negative. changes. Hyperparakeratosis was observed in 13 biop- sies and orthokeratosis in three. Acanthosis was found in 10 lesions while epithelial atrophy was observed in Discussion two red maculae. Vacuolar degeneration of the base- In the present study, oral lesions of patients with SLE ment membrane with necrosis of basal keratinocytes was and CLE were characterized. This is the first Venezuelan A B C