Topical rapamycin versus betamethasone dipropionate ointment for treating oral erosive lichen planus: a randomized, double-blind, controlled study.

BACKGROUND: Although superpotent topical corticosteroids are the first-line treatment for oral erosive lichen planus (OELP), topical rapamycin was found efficient in a previous case series. OBJECTIVES: To compare the efficacy and safety of topical rapamycin and betamethasone dipropionate ointment for OELP in a randomized, double-blind trial. METHODS: Patients were randomized to receive treatment with betamethasone dipropionate ointment 0.05% in Orabase® or topical rapamycin solution (1 mg/mL) on lesions twice daily for 3 months, followed by 3 months of observation. The primary outcome was clinical remission after 3 months of treatment. Secondary outcomes were clinical remission after 1 and 2 months, reduced oral pain and reduced impact on food intake after 3 months, clinical recurrence after treatment withdrawal, and adverse events. RESULTS: During a 4-year period, 76 patients were randomized and 75 received treatment (rapamycin, n = 39; betamethasone, n = 36). At 3 months, 39.4% of patients with betamethasone and 27.3% with rapamycin showed clinical remission (odds ratio 0.68, 95% CI [0.24; 1.89]; P = 0.46). Rates of remission after 1 and 2 months, reduction in pain and impact on food intake after 3 months, were higher with betamethasone than rapamycin. Recurrence of oral erosions was similar between groups. Adverse events occurred in 43.6% of patients with rapamycin (mostly burning sensation, impaired taste) and 27.8% with betamethasone (mostly oral candidiasis). CONCLUSION: Although the study was limited by insufficient recruitment, we did not find any superiority of topical rapamycin over betamethasone dipropionate ointment for OELP. Given the rapid remission and pain improvement in the betamethasone group, it appears that superpotent topical corticosteroids should remain the first-line treatment for OELP.

Rituximab as a trigger factor of medication-related osteonecrosis of the jaw. A case report.

Rituximab, an anti-CD20 monoclonal antibody, is an effective treatment for rheumatoid arthritis. Here we report the case of a patient with rheumatoid arthritis, having taken risedronate for 14 months to prevent corticosteroid-induced osteoporosis, more than 2 years ago, who presented osteonecrosis of jaw following herpetic gingivostomatitis two weeks after the beginning of a rituximab treatment associated with her usual anti-rheumatic drugs. Eight weeks later, no bone and/or gum healing was observed and a stage 2 medication-related osteonecrosis of the jaw (MRONJ) was diagnosed. A conservative approach was decided with antiseptic mouth washes, low-level laser treatment (LLLT) and systemic therapy with teriparatide. Complete mucosal coverage was obtained after more two years of follow-up. We suggest that rituximab as immunosuppressant might be a cause or a decompensating factor of MRONJ. Non-surgical periodontal treatment with LLLT and teriparatide are candidates for the treatment of MRONJ.

[Oral care recommendations for patients with oral autoimmune bullous diseases]. / Recommandations pour la prise en charge buccodentaire des patients atteints de maladies bulleuses auto-immunes avec atteinte buccale.

BACKGROUND: Autoimmune bullous diseases (AIBD) may cause chronic oral lesions that progress insidiously. AIMS: To provide recommendations for optimal oral-dental management of patients presenting AIBD with oral involvement. PATIENTS AND METHODS: In the absence of scientific studies with high levels of proof, these recommendations have been drawn up at two meetings by a committee of experts on AIBD comprising 7 dermatologists, 1 stomatologist, 1 maxillofacial surgeon, 2 odontologists and 4 parodontologists. RESULTS: The oral lesions associated with AIBD may be classified into three grades of severity: severe (generalised erosive gingivitis affecting at least 30% of dental sites), moderate (localised erosive gingivitis affecting less than 30% of dental sites) and controlled (no erosive oral lesions). Good oral-dental hygiene suited to the severity of the oral lesions, must be practised continually by these patients so as to avoid the formation of dental plaque, which aggravates symptoms. Dental and parodontal care must be considered in accordance with the severity grade of the oral lesions: in severe cases, the dental plaque must be eliminated manually with a curette, but several types of care (descaling, treatment for tooth decay, non-urgent extractions, etc.) must be suspended until the grade of severity is moderate or until the disease is stabilised.

A hair on the tongue.

INTRODUCTION: Human hairs are generally localized on the cutaneous part of the head, neck, torso, armpits, pubis and limbs. Sometimes it can be found in an unusual localization and is then called heterotopic. OBSERVATION: A 30-year-old man presented with a hair in the middle of the dorsum of the tongue. It was decided to perform an excision under local anesthesia. DISCUSSION: Few reports exist that describe hair growing on mucosa. Only one other case has been published concerning the tongue.

[Mucosal lymphomatoid papulosis: 2 cases]. / Papulose lymphomatoïde de localisation muqueuse : deux cas.

BACKGROUND: Lymphomatoid Papulosis (LP) is a chronic dermatosis progressing by flare-up. According to the WHO-EORTC classification, LP is a form of CD30+ primitive cutaneous lympho-proliferation. Mucosal lesions are rare, with 15 published cases. We report two new cases of oral localizations, without any cutaneous involvement. PATIENTS AND METHODS: Two women, 32 and 63 years old, presented with an isolated painful oral ulceration, of the maxillary tuberosity and of the inner side of the cheek respectively. The general state of health was preserved. Immunohistochimical analysis of the biopsies showed two Type A LPs. Lesions spontaneously resolved. DISCUSSION: Among the rare published cases, oral localization involved exclusively the tongue and the labial mucosa and almost all patients presented with previous cutaneous lesions. Isolated maxillary tuberosity or cheek involvements were not described yet.

Treatment of refractory oral erosive lichen planus with topical rapamycin: 7 cases.

BACKGROUND: Chronic erosive oral lichen planus (CEOLP) is a painful disease. Topical steroids constitute the mainstay of treatment. Given the reports of a slightly greater risk of squamous-cell carcinoma, rapamycin may be a good candidate for recalcitrant CEOLP, as it has both immunosuppressive and antitumour properties. OBJECTIVES: To investigate the therapeutic effect and evaluate the blood absorption of topical rapamycin in patients with CEOLP. PATIENTS AND METHODS: We carried out an open prospective study: 7 women with CEOLP applied topical rapamycin (1 mg/ml) on oral erosive lesions twice a day for 3 months. Four patients also had erosive vulvar lesions and applied the same solution on both mucosae. We monitored blood sirolimus levels 15 days after the initiation of treatment. Complete remission was defined by the disappearance of oral erosions and partial remission when the surface of oral erosions was 50% less than the surface of the initial erosion. RESULTS: At 3 months, 4 women had complete remission and 2 women had partial remission. One patient stopped treatment due to local discomfort. Only 1 woman had blood sirolimus levels that were detectable. CONCLUSION: Topical rapamycin may be effective in some cases of refractory CEOLP, with negligible absorption into blood and minimal side effects.

Melkersson-Rosenthal syndrome and acquired C1 inhibitor deficiency.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease whose full-blown form is characterized by orofacial swelling, facial palsy and lingua plicata. OBJECTIVE: To investigate the complement system as well as its role in patients with MRS. METHODS: Seven patients presenting at this hospital between November 2002 and May 2003 and meeting the diagnostic criteria according to Hornstein were evaluated retrospectively. The investigations included clinical signs, an analysis of the complement system including levels of CH50, C3, C4, C1 inhibitor (INH) functions and C1-INH antigen detection. RESULTS: Two female patients showed isolated low levels of functional C1-INH as determined by duplicate tests. Both patients took estrogen-progestin contraceptives. CONCLUSION: Since deficiency in plasma protease C1-INH is known to lead to recurrent angioedema, we hypothesize that low levels of functional C1-INH may have contributed to the orofacial swelling in the 2 patients.

[Cicatricial pemphigoid: management in stomatology]. / Pemphigoïde cicatricielle: prise en charge en stomatologie.

BACKGROUND: Cicatricial pemphigoid is a subepidermal disease of the mucous membranes. In some patients blisters involving the oral cavity may be the only manifestation. The purpose of this work was to detail the clinical features and biological findings, highlighting the diagnostic and therapeutic difficulties. MATERIAL AND METHODS: Seventeen patients, nine women (mean age 59.6 years) and eight men (mean age 67.8 years) managed between 1990 and 2000 were reviewed retrospectively. RESULTS: Mucous membranes of the mouth were the only localization in six of the seventeen patients. Other localizations (skin, eye, nose, anus) were associated, alone or in combination, in the other eleven patients. The gingivae were involved in 15/17 patients and constituted the only localization in six. Skin lesions (sometimes involving the lower limb) were variable and found in 6/17 patients. Ocular lesions were present at diagnosis in six patients, including two with severe lesions at onset. Nasal and/or laryngeal involvement was found in 6/17 patients. These lesions were often severe with major functional impact. ENT localizations were found in patients with at least three localizations and lesions extending to mucous membranes distant from the oral cavity. A biopsy was obtained from all patients and demonstrated subepidermal detachment in eleven. Direct immunofluorescence was positive in eleven. Indirect immunofluorescence, performed in twelve patients, was negative. In two patients, both histology and immunofluorescence were negative. Electron immunomicroscopy was performed in four patients and confirmed the diagnosis. Five out of six isolated oral manifestations were treated with topical steroids alone. Multiple lesions were treated case by case using daprosone and/or systemic corticosteroids associated with topical steroids in 16/17 patients. A bolus of cyclophosphamid was given for four patients with very severe lesions which did not respond to other treatments. At last follow-up (22 +/- 17 months), none of the patients was considered cured. DISCUSSION: Cicatricial pemphigoid is a complex chronic disease. Oral manifestations may be isolated or associated with lesions of the skin or other mucous membranes. The painful aspect of oral lesions explains why the diagnosis is often made in stomatology. Careful evaluation at diagnosis is essential and requires a multidisciplinary approach. Certain diagnosis can be provided by immunohistology, particularly electron immunomicroscopy which enables distinguishing cicatricial pemphigoid from other autoimmune diseases of the dermo-epidermal junction. Treatment depends on disease severity. Isolated oral manifestations are generally well controlled by topical corticosteroids which ocular, nasal, and laryngeal localizations often require more extensive treatment.

[Biopsy of the accessory salivary glands. 5 years' experience]. / Biopsie des glandes salivaires accessoires. Bilan de 5 ans.

Biopsy of the minor salivary glands has become a routine examination ordered by physicians working in a wide range of disciplines in order to search for or eliminate the diagnosis of Gougerot Sjögren's disease or another systemic disease. We emphasize the need to use this examinations as a part of a complete work-up of the buccal cavity and the salivary glands. We reviewed our experience with 1,500 biopsies. The glands biopsied were normal in 56% of the cases and led to the diagnosis of Gougerot Sjögren's disease in 24%, chronic sialadenitis in 10% and diverse trophic problems in 5%. The clinical stage of Gougerot Sjögren's disease is usually proposed according to the Chisholm classification which we propose to compare with the Chomette classification. Finally, we described the technique of minor salivary gland biopsy.

[Histopathologic lesions of the accessory salivary glands in Gougerot-Sjögren syndrome: re-evaluation of the diagnostic criteria of Chisholm and Mason and of Chometter et al]. / Lésions histopathologiques des glandes salivaires accessoires (G.S.A.) au cours du syndrome de Gougerot-Sjögren: réévaluation des critères diagnostiques de Chisholm et Mason et de Chomette et coll.

In a retrospective study, we used a series of 29 patients with Sjögren's Syndrome diagnosed according to the new European criteria (Cl. Vitali, S. Bombardieri, H. M. Moutsopoulos et al.) (8). (Arthritis Rheum 1993; 36:340-7). A labial biopsy technique allowed to classify these patients into the anatomical "scores" formerly related by Chisholm and Mason and Chomette et al. Referring to these criteria, only 45% of patients presented a characteristic histopathological pattern. If another complementary criterium, i.e. ductal tropism of lymphoid infiltrates, was added, that percentage remained low (50% only). Thus, these results would suggest the following considerations: the classical histopathological criteria do not seem sufficiently specific; other histological criteria such as ductal lesions previously noted by Leroy et al. must in addition be looked for; thus it would seem to be of considerable value to use in the future complementary quantitative studies by means of morphometric methods.

[Lip ulceration in paraneoplastic dermatomyositis. Apropos of a case. Review of the literature]. / Ulcération labiale au cours d'une dermatomyosite paranéoplasique. A propos d'un cas. Revue de la littérature.

A 71 year old woman with metastatic ovarian cancer developed ulceration of the lips during the course of a paraneoplastic dermatomyositis. The principal diagnostic features of multiple myositis are described, as well as the relationship of buccal lesions to the dermatomyositis. Data are lacking on the frequency of these polymorphous and non specific lesions.