Miescher's cheilitis granulomatosa. A presentation of five cases

CAMACHO-ALONSO F, BERMEJO-FENOLL A, LÓPEZ-JORNET P. MIESCHER'S CHEILITIS GRANULOMATOSA. A PRESENTATION OF FIVE CASES. MED ORAL PATOL ORAL CIR BUCAL 2004;9:425-9.

SUMMARY

Miescher's cheilitis granulomatosa (CG) consists of the appearance of recurrent labial edema on one or both lips, which can become persistent. It has traditionally been considered as a monosymptomatic form of the Melkersson-Rosenthal syndrome, described as the association of recurrent labial and/or recurrent facial edema, relapsing facial paralysis and fissured tongue.
The aim of this study is to present a series of five clinically and histopathologically diagnosed cases of CG that came to our clinic at the Teaching Unit of Oral Medicine, Faculty of Medicine and Odontology, University of Murcia. A complete study of these patients evaluated the age, sex, family history, and location and course of the signs and symptoms. Various complementary examinations were carried out, studying the hematic characteristics (hemogram, erythrocyte sedimentation rate, leukocyte count), including immunological and histopathological studies.
The treatment consisted of intralesional corticoids, combined in some cases with anti-leprous drugs or systemic corticoids. A good response to treatment was obtained in all cases.

Keywords: Cheilitis granulomatosa, Miescher's cheilitis, Melkersson-Rosenthal syndrome.

INTRODUCTION

Cheilitis granulomatosa is an infrequent illness, characterised by the appearance of a recurrent labial swelling that corresponds to a non-specific granulomatous reaction in one or both lips, which can become persistent. It was first described by Miescher in 1945, and has traditionally been considered as a monosymptomatic form of the Melkersson Rosenthal syndrome (MRS), described as the association of recurrent labial edema and/or recurrent facial edema, relapsing facial paralysis and fissured tongue.

The appearance of the classic triad of MRS is infrequent however, appearing in approximately 25 to 40 per cent of cases. Generally, patients present oligosymptomatic or monosymptomatic forms of the illness, cheilitis granulomatosa being the most common monosymptomatic form (1-5).

The purpose of this study is to present our experience in the management of five patients with Miescher's cheilitis granulomatosa, and to compare the results obtained with those existing in the literature.

CLINICAL CASES

We present a group of five patients (four female and one male) with monosymptomatic forms of MRS who attended our clinic at the Teaching Unit of Oral Medicine, Faculty of Medicine and Odontology, University of Murcia, during the period between January 1985 and September 2002. All patients presented persistent labial tumefaction, which did not exceed 10 months evolution in any case. The age range of this group was from 17 to 66 years. In each case the age and sex, location and development of the signs and symptoms presented, and likewise the possible existence of a family history of labial tumefaction were evaluated. A complete analysis was requested for all patients and a biopsy of the affected lip was taken. The sections were stained with haematoxylin-eosin and the principal histopathological characteristics were studied. Finally, we recorded the therapeutic treatments applied to each patient, and their corresponding efficacy in the reduction of the labial edema.

Table 1 shows the principal characteristics of each case. The average age of our five patients was 36 years (range 17-66), being most frequent in women (4 out of 5 cases). All patients presented labial tumefaction that had begun in an insidious and asymptomatic manner (Fig.1).

The average period of evolution of the labial inflammation at the time of treatment was 6.8 months (range 2-10), remaining without remittance in all the patients throughout this period.

No family history of orofacial edema was found in any of the cases. The upper lip was the most frequently affected area, tumefaction in both lips being observed in only one patient, who also presented left hemifacial edema, and fissured tongue (Table 1). The hemogram, erythrocyte sedimentation rate, leukocyte count, and IgA levels were found to be within normal limits in all patients. The histopathological study of the biopsied samples (Figs. 2 and 3) revealed a granulomatous inflammation in all cases (Table 1).Four patients were treated with intralesional corticoid infiltration (triamcinolone 1 cm³ every 15 days for 45 days). In one patient this was as the exclusive treatment, in two patients in combination with clofazimine (one 100 mg capsule every 48 hours), and in one other together with systemic betamethasone (in capsules of 4 mg per day). The remaining patient was treated with clofazimine only. A positive response to treatment was obtained in all cases, observing a notable reduction in the inflammation by the end of the treatment.

DISCUSSION

MRS is an illness characterised by the presence of recurrent facial edema and/or recurrent labial edema, relapsing facial paralysis and fissured tongue. However, the presence of the classic triad is infrequent, being found in around 25 to 40 per cent of cases (4). The oligosymptomatic and monosymptomatic forms of the illness are the most frequent, and amongst these the most common is Miescher's cheilitis granulomatosa.

The etiology of MRS is unknown, although currently three possible origins of the illness are considered: 1- the infectious (6), 2- the immunological, triggered off by allergy or intolerance to some foods (7), and 3- a possible genetic origin (8).

Females seem to be affected slightly more than males, although the figures in this respect are confusing (5, 9). The majority of patients experience the first symptoms in the second decade of life, with a wide range in relation to the age at presentation (from 6-63 years) (5). In our cases the average age was 36 years, with a majority of females being affected (4 out of 5).

Recurrent labial edema is the first manifestation of MRS in a high percentage of patients (43%), and appears at some time during the clinical course of the process in the majority of cases (84%) (5). We observed this fact in all our five patients, and furthermore, finding one case that also presented left hemifacial edema and fissured tongue. As can be observed in Table 1, in our patients the inflammation of only one lip was more frequent (4 out of 5) than both, the upper lip being the one most affected, results which coincide with those obtained by other authors (10, 11).

The diagnosis is fundamentally clinical and is confirmed with a biopsy. The hemogram can present increased values for the erythrocyte sedimentation rate, lymphocytosis, peripheral eosinophilia, and on some occasions an increase in the IgA (12). None of these alterations appeared in any of our patients. A differential diagnosis should be established with all those processes that frequently occur with facial edema (both intra and extra bucal) and other granulomatous illnesses.

The histopathology of MRS is characterised by the appearance of a granulomatous inflammation in a high percentage of cases (67%), although the appearance of granulomas does not necessarily indicate the existence of the syndrome, as they can appear simply as a non-specific inflammatory reaction (9). In the five cases presented, the inflammation was granulomatous.

Numerous drugs have been used for the treatment of oral facial edema: systemic antibiotics, salazosulfapyridine, radiotherapy; and systemic, intralesional, and topical corticoids. Some authors (13) have used anti-leprous drugs, such as clofazimine, in patients with MRS, achieving a reduction in the labial edema in 94 per cent of cases. In our cases we applied systemic and intralesional corticoids and clofazimine, obtaining satisfactory results in all cases. On occasions, the use of plastic surgery is necessary when the pharmacological treatment proposed above is not able to reduce the labial deformities completely (14).

Finally, we highlight the importance of evaluating these patients using a multi-disciplinary team, which besides dentists, should also include: dermatologists, neurologists, otolaryngologists, pathologists and plastic surgeons.

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