CASO CLÍNICO

Melkersson-Rosenthal syndrome: case report and literature review

Síndrome de Melkersson Rosenthal: reporte de un caso y revisión de la literatura

R. Cosmelli Maturana¹, R. Bravo Ahumada², L. Cordova Jara²

¹ Cirujano Dentista. Meritante Equipo de Cirugía Maxilofacial.
² Cirujano Maxilofacial. Equipo de Cirugía Maxilofacial.
Complejo Hospitalario San José, Santiago de Chile, Chile

Correspondence

ABSTRACT

Melkersson-Rosenthal syndrome is described as the association of recurrent lip edema (Miescher’s cheilitis granulomatosa) and/or facial edema, recurrent facial paralysis, and plicated tongue. Recurrent edema occurs in one or both lips, and can be persistent. When lip edema alone occurs, the case is considered a monosymptomatic form of this syndrome. The aim of this review was to discuss the follow-up of a patient seen in our department with the diagnosis of Melkersson-Rosenthal syndrome who presented intense cheilitis granulomatosa associated with plicated tongue. The patient was treated with infiltration of intralesional corticoids and later cheiloplasty.

Key words: Melkersson-Rosenthal syndrome; Cheilitis granulomatosa; cheiloplasty.

RESUMEN

El Síndrome de Melkersson Rosenthal se describe como la asociación de edema labial (queilitis granulomatosa de Miescher) y/o facial recurrente, parálisis facial recidivante y lengua fisurada. El edema labial recurrente se presenta en uno o ambos labios, el cual puede llegar a ser persistente. Cuando se presenta en forma aislada se considera como una forma monosintomatica de este síndrome. El objetivo de esta revisión es mostrar el seguimiento de un caso clínico atendido en nuestro servicio con el diagnóstico síndrome de Melkersson Rosenthal que presenta una gran queilitis granulomatosa asociada a lengua fisurada, cuyo tratamiento consistió en la infiltración de corticoides intralesionales y una posterior queiloplastía.

Palabras clave: Síndrome de Melkersson Rosenthal; Queilitis granulomatosa; Queiloplastía.

Introduction

Melkersson-Rosenthal syndrome (MRS) is a rare pathology of unknown origin that is characterized by three clinical signs: recurrent labial and/or facial inflammation, plicated tongue, and facial paralysis.^1-3 Exacerbations and recurrence are common in the syndrome. The orofacial swelling is characterized by inflamed, fissured lips of a reddish brown color.

The presentation of the classic triad of MRS is uncommon, occurring in approximately 25-40% of cases.² The condition generally presents as an oligosymptomatic or monosymptomatic form, with cheilitis granulomatosa being the most common monosymptomatic form.^2,4 Cheilitis granulomatosa (CG) was described by Miescher in 1945.⁴ It is characterized by the appearance of recurrent labial swelling produced by a nonspecific granulomatous reaction in one or both lips, which may eventually become persistent. The size of the lower lip generally increases, consisting of uniform swelling that may be associated with other intraoral swelling, especially of the palate and mouth floor. In addition to the esthetic problem, the enlarged and disfigured lip usually gives the patient difficulties when eating, drinking, and speaking.^3,5

The diagnosis of MRS is completed by the histologic finding of noncaseifying, sarcoid type granulomas, although their presence is not constant. The absence of this symptom, however, does not exclude the diagnosis of MRS if other signs are present.

The clinical manifestations of this syndrome are manifested in 50% of cases after the age of 20 years 6,7 and MRS shows a predilection for women.^2,8

The treatment of this pathology is controversial because its etiopathogenic mechanism has not been fully explained. Different treatments have been tried with variable results. Therapeutic options range from the systemic and intralesional use of corticoids, alone, or combined with other drugs to increase their effectiveness. Surgical treatment has been resorted to in residual cases and there have been reports of recurrence.

The current study presents a clinical case of a woman seen in our department with a diagnosis of MRS and intense CG associated with plicated tongue. Her treatment consisted of intralesional infiltration of corticoids and later cheiloplasty.

Clinical case

A 24-year-old woman was seen in the Maxillofacial Surgery Department of San José Hospital Complex in October 2006, referred from the Dermatology Department of the same center with a diagnosis of MRS. Her increased lower lip volume had not responded to treatment with intralesional and oral corticoids.

The patient stated that her lower lip began to increase in volume in 2004. A biopsy performed that year yielded a histopathologic diagnosis of CG associated with MRS. The initial treatment consisted of intralesional infiltration performed in the dermatology department with betamethasone 4 mg and prednisone 0.5 mg, which failed to produce satisfactory results.

In the interview, the patient related a medical history of epilepsy, which was under treatment, mild mental retardation, hypothyroidism, insulin resistance, and iodine allergy. She regularly took levothyroxine 100 mg, 1 tablet/day, carbamazepine 200 mg, 1.5 tablet/day, metformin 850 mg, 1 tablet/day.

The physical examination disclosed a large increase in the volume of the lower lip and lip eversion (Figs. 1 and 2), which were associated with depapillation and transversal furrows on the dorsal tongue consistent with plicated tongue, as well as discrete macroglossia (Fig. 3).

Treatment consisted of 3 intralesional infiltrations in the lower lip of 1 ml Kenacort^®-A 40 mg (triamcinolone acetonide) diluted in 1 ml of lidocaine 2% to control postinfiltration pain, which were administered at one-week intervals. The patient was followed up for three months, during which the size of the lower lip decrease markedly, but without achieving an esthetically acceptable result. A fourth infiltration was given but no improvement was observed (Figs. 4 and 5). A lower-lip-reduction cheiloplasty was performed under general anesthesia to improve the appearance of the patient’s lip. The results were good one month after surgery (Figs. 6 and 7).

Discussion

MRS is a disease constituted by a clinical triad consisting of facial or labial edema, recurrent facial paralysis, and plicated tongue. CG is the most common monosymptomatic form of MRS.¹

The etiology of MRS is not known, but three possible origins of the disease are being discussed: infection,⁹ an immunologic reaction triggered by allergy or food intolerance,⁹ and a possible genetic component.^10,11 Given the lack of a clear etiology, the treatments have been diverse and numerous topical and systemic drugs have been used: systemic antibiotics, salazosulfapyridine, irradiation, topical, intralesional and systemic corticoids, and antileprosy drugs. Cheiloplasty often is associated to enhance results.^12,13 Nevertheless, the results have not always been satisfactory.

The use of steroids has produced good results in improving most of these patients.¹⁴ We gave this patient triamcinolone acetonide, a corticoid with a powerful and prolonged anti-inflammatory, hormonal, and metabolic effect. Although triamcinolone is approximately 1 to 2 times stronger than prednisone in animal models of inflammation, the derivative known as triamcinolone acetonide is even stronger, being approximately 8 times more potent than prednisone.¹⁵ The necessary precautions to be considered with this drug include those related with its prolonged duration and potency. Total adrenal suppression has been reported with a single intramuscular infiltration of 60 to 100 mg of triamcinolone acetonide, which appears 24 to 48 hours after infiltration; adrenal function recovers gradually over 30 to 40 days. Consequently, we started therapy with a maximum infiltration of 40 mg of triamcinolone acetonide, once weekly, for three weeks. The results were acceptable, but not optimal, so a new intralesional infiltration of the same drug was made at three months. However, further labial reduction was not achieved and a reduction cheiloplasty was performed.

Multidisciplinary cooperation is important in the management of these patients because no standardized therapy exists. Consensus is still lacking in the literature on the etiopathogenic mechanisms involved. Cases and series of cases with a variety of treatments and a range of results have been reported.

Patients with MRS probably will experience a recurrence of symptoms in the course of their lives because the therapeutic alternatives usually do not provide any definitive solution.

Correspondence:
Dr. Rodrigo Cosmelli Maturana.
Complejo Hospitalario San José,
Santiago-Chile
rcosmelli@gmail.com

Recibido: 31.03.2008
Aceptado: 16.06.2008

References

1. Stein SL, Mancini AJ. Melkersson-Rosenthal syndrome in childhood: Successful management with combination steroid and minocycline therapy. J Am Acad Dermatol 1999;41:746-8.        [ Links ]

2. Zimmer WM, Rogers RS, Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from literature. Oral Surg Oral Med Oral Pathol 1992;74:610-9.        [ Links ]

3. Sapp JP, Eversole LR, Wysocki GP. Patología Oral y Maxilofacial Contemporánea. 2ª ed. Mosby, 2005 Edición en Español. Elsevier España, S.A.        [ Links ]

4. Miescher G. Ubre essentielle granulomatöse macrokelie (cheilitis granulomatosa). Dermatológica 1945;91:57-85.        [ Links ]

5. Camacho-Alonso F, Bermejo-Fenoll A, López-Jornet P. Queilitis granulomatosa de Miescher. Presentación de cinco casos. Med Oral Patol Oral Cir Bucal 2004; 9:425-9.        [ Links ]

6. Alexander RW, James RB. Melkersson-Rosenthal syndrome: review of literature and report of case. J Oral Surg 1972;30:599-604.        [ Links ]

7. Worsaae N, y cols. Melkersson-Rosenthal syndrome and cheilitis granulomatosa. A clinicopathological study of thirty-three patients with special reference to their oral lesions. Oral Surg Oral Med Oral Patho 1982;l54:404.        [ Links ]

8. Greene RM, Rogers RS III: Melkersson-Rosenthal syndrome: A review of 36 patients. J Am Acad Dermatol 1989;21:1263.        [ Links ]

9. Ang KL, Jones NS. Melkersson-Rosenthal Syndrome. J Laryngol Otol 2002;116:386-8.        [ Links ]

10. Gerressen M, et al. Melkersson-Rosenthal syndrome: case report of a 30-Year Misdiagnosis. J Oral Maxillofac Surg 2005;63:1035-9.        [ Links ]

11. Shapiro M, Peters S, Spinelly HM. Melkersson-Rosenthal Syndrome in the periocular area: A Review of the literature and Case Report. Ann Plast Surg 2003; 50:644-8.        [ Links ]

12. Ellitsgaard N, Andersson AP, Worsaae N, Medgysei S. Long-term results after surgical reduction cheiloplasty in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Ann Plast Surg 1993;31:413-20.        [ Links ]

13. Cederna PS, y cols. Melkersson- Rosenthal Síndrome: Reduction Cheiloplasty Utilizing a Transmodiolar Labial Suspension Suture. Plastic Surgery. Aesthetic Plastic Surg 1998;22:102-5.        [ Links ]

14. El-Hakim M, Chauvin P. Orofacial Granulomatosis Presenting as Persistent Lip Swelling: Review of 6 New Cases. J Oral Maxillofac Surg 2004;62:1114-7.        [ Links ]

15. Hernecki J, Sararols L. Triamcinolona acetónido en el tratamiento del edema macular diabético. Annals d’Oftalmologia 2004;1:71-6.        [ Links ]