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Vol. 26. Issue 6.
Pages 291-293 (November 1998)
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Vol. 26. Issue 6.
Pages 291-293 (November 1998)
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Melkersson-Rosenthal syndrome in a diabetic boy.
Melkersson-Rosenthal syndrome in a diabetic boy.
E. Ruza Paz-Curbera
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ALLERGOL. ET IMMUNOPATHOL., 1998;26(6):291-293

Melkersson-Rosenthal syndrome in a diabetic boy

E. Ruza Paz-Curbera* and M. Fernández Benítez

University Clinic of Navarra. Faculty of Medicine. Pamplona, Spain. Department of Allergology and Clinical Immunology. *Department of Paediatrics.


The Melkersson-Rosenthal syndrome (MRS) consists of recurrent edema of the lips, intermittent facial palsy and furrowed tongue. This is the classic triad which defines the syndrome, although it is accepted that the presence of two manifestations or one with a granulomatous cheilitis in the biopsy, are sufficient to make the diagnosis. The case of a 15 year-old diabetic boy is presented. He had a persistent edema of the upper lip of one-year duration, which started abruptly without any clear etiologic correlation. He was treated with antihistaminics and corticosteroids without improvement but with diabetic imbalance. The complementary examinations were normal and the biopsy was compatible with granulomatous cheilitis.

Despite the low incidence and the fact that there is no specific treatment, MRS has to be considered as a diagnostic possibility in a patient with recurrent edema although not necessarily having the complete triad.

Key words: Melkersson-Rosenthal syndrome. Diabetic boy. Labial edema. Granulomatous cheilitis.

Allergol et Immunopathol 1998;26:291-3.


Melkersson syndrome was described in 1928 as peripheral facial palsy and labial edema. Rosenthal, in 1930, included the presence of furrowed tongue, completing the clinic triad which defines the syndrome. However, the complete triad is not common and it is accepted that the presence of at least two clinic criteria are enough to make the diagnosis (1).

Miescher, in 1945, described from an antomophatologic point of view the monosymptomatic form of recurrent labial edema as a granulomatous cheilitis (2, 3).

A review of the literature reports published shows that its low incidence in general population, and specially in childhood (4), and the variable clinical presentation can make its diagnosis difficult.


We present a 15 years-old boy suffering from insulin-dependent diabetes mellitus for 3 years. He suddenly presented a non-pruritic, painless, hard and persistent upper lip edema which lasted one year. Some months afterwards, right cheek edema was associated. He did not refer any triggering factors and personal or family history of atopy.

He went to an allergologist in another centre, who found only a weak positivity in the skin test to Dermatophagoides pt. not confirmed by the in vitro methods.

He then followed treatment with antihistaminics and short courses of corticosteroids without improvement at all, but developing an important diabetic imbalance.

His doctor recommended him the extraction of his lower right molar and antibiotic treatment, without improvement.

He came to our centre. On physical examination, he presented a fissured upper lip edema involving his right cheek. The right oral mucosa appeared swollen and like a "paving" (Figs. 1 and 2).

Figure 1.--Marked edema of upper lip and right cheek, persistent during one year.

Figure 2.--Right oral mucosa appeared swollen and like a "paving".

The complementary explorations were into normal ranges. Hemogram, VSG, IgE, cortisol and ACTH, cholesterol and fractions, urea, creatinine, iron and antinuclear, antimythocondrial and smooth muscle antibodies were negative. The study of the cellular immune system was not conclusive.

We performed skin tests (intradermal tests) to the most common inhalant allergens (molds, pollens, mites, animal danders) and foods (fish, seafood, legumes, cow''s milk, wheat, fruit, vegetables and nuts). We only found a weak positive response to Dermatophagoides pt. Serum specific IgE determination (CAP-FEIA, Pharmacia, Sweden) and histamine release test did not confirm the intradermal test result.

The paranasal sinuses and thorax radiological study, and the colonoscopy with biopsy did not show pathologic findings.

Finally, a lip biopsy was perfomed. The anatomopathologic study revealed a diffused and perivascular lymphoplasmocitary infiltrate, with aggregates of epitelioids cells, compatible with granulomatous cheilitis.


The differential diagnosis of recurrent lip edema in childhood can be difficult. The sudden beginning, the persistent evolution, the negative family history and the antihistaminic and corticosteroid treatment without improvement suggested the MRS diagnosis.

Zimmer et al. (5) reviewed 42 patients with MRS and compared them to 220 cases described in the European literature published between 1965-1990. In 81% of the cases, the oropharyngeal manifestations constituted the initial sign, being lip edema the predominant symptom. During the course of this illness, 74% of patients developed lip edema, and only 35% presented neurologic manifestations being the peripheral facial palsy the most frequent. Others as migraine, vertigo, vasomotor dysfunction, etc. were described.

In the last years, MRS is being considered as part of a new and broader entity called orofacial Granulomatosis (6-9), which also includes sarcoidosis, food allergy, dental infections and oral Crohn, in fact this last form can be the initial manifestation of a bowel Crohn disease.

At this moment, the association between MRS and diabetes mellitus has not been described.

Today, there is no specific treatment for MRS. Different drugs as thalidomide, hidroxicloroquine, dapsone, colchicine (10, 11) have been used with non concluding results. Systemic and/or intralesional corticosteroids were considered the election treatment, but recent reports showed contradictory results about their efficacy (10, 12).

In the last years, several reports described the employ of clofazimine showing encouraging results, even though more studies are necessary to verify its therapeutic efficacy (3, 10, 13-15).

In severe cases which do not respond to medical treatment, reductory cheiloplasty can be the election treatment with esthetic results (10).

Rare cases of spontaneous remissions have been reported (13).


MRS has to be considered in every patient presenting persistent and/or recurrent lip edema, being or not associated with neurologic and/or dermatologic features.

Anatomopathologic confirmation of granulomatous cheilitis might help to confirm the diagnosis in monosymptomatic or oligomonosymptomatic forms.


El síndrome de Melkersson-Rosenthal se define como edema labial recurrente asociado a parálisis facial periférica y lengua escrotal. Sin embargo, no es frecuente la presentación completa de esta tríada clínica y se acepta que, la existencia de al menos dos criterios clínicos o uno de ellos junto con la existencia de una queilitis granulomatosa en la biopsia, son suficientes para establecer su diagnóstico.

Presentamos el caso clínico de un varón de 15 años afecto de diabetes insulin-dependiente, que de forma súbita comenzó con un edema de labio superior, no pruriginoso ni doloroso, indurado y persistente durante un año, que posteriormente se extendió a mejilla derecha. No refería antecedentes personales ni familiares de atopia, ni correlación etiológica clara. Realizó tratamiento con antihistamínicos sin mejoría, y con pautas cortas de corticoides que, aunque mejoraban de forma parcial el edema, producían a su vez una importante descompensación de su diabetes.

En el estudio alergológico realizado, se observó una positividad cutánea en los tests intradérmicos realizados frente a los alergenos inhalantes y alimentarios más frecuentes, que no fue confirmada por IgE específica ni por el test de liberación de histamina. Sin embargo, la biopsia del labio afectado mostró la existencia de una queilitis granulomatosa.

Una revisión de los casos publicados en la literatura muestra la baja incidencia de la presentación sindrómica completa, siendo el estudio anatomo-patológico fundamental para orientar el diagnóstico en las formas mono u oligosintomáticas y para profundizar en una nueva entidad denominada granulomatosis orofaríngea.

Palabras clave: Síndrome de Melkersson-Rosenthal. Niño diabético. Edema labial. Queilitis granulomatosa.


1. Moreno MV, Fernández M, Oehling A. Melkersson-Rosenthal syndrome. Allergol et Immunopathol 1988;16:369-72.

2. Grosshans E, Pfeffer S. Le syndrome de Melkersson-Rosenthal. La macrocheilite granulomateuse de Miescher. Ann Dermatol Venereol 1991;118:245-51.

3. Marques C, Machado A, Baptista AP. Macroqueilities e sindrome de Melkersson-Rosenthal. Revisao de 19 casos. Acta Med Port 1994;7:533-40.

4. Cohen HA, Cohen Z, Ashkenasi A, Straussberg R, Frydman M, Kauschansky, et al. Melkersson-Rosenthal syndrome. Cutis 1994;54:327-8.

5. Zimmer WM, Rogers RS, Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. Oral Surg Oral Med Oral Pathol 1992;74:610-9.

6. Rogers RS. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371-9.

7. Sabroe RA, Kennedy CT. Facial granulomatous lymphoedema and syringomielia. Clin Exp Dermatol 1996;21:72-4.

8. Yeatts RP, White WL. Granulomatous blepharitis as a sign of Melkersson-Rosenthal syndrome. Ophtalmology 1997;104: 1185-90.

9. Mahler VB, Hornstein OP, Boateng BI, Kiesewetter FF. Granulomatous glossitis as an unusual manifestation of Melkersson-Rosenthal syndrome. Cutis 1995;55:244-8.

10. Rey R, Carreau JP, Gola R, Berbis P. Syndrome de Melkersson-Rosenthal. Interet de la cheiloplastie de reduction. Ann Dermatol Venereol 1996;123:325-7.

11. Safa G, Joly P, Boullie MC, Thomine E, Lauret P. Syndrome de Melkersson-Rosenthal traite par le thalidomide. Deux observations. Ann Dermatol Venereol 1995;122:609-11.

12. Daoud MS, Rogers RS. Melkersson-Rosenthal syndrome. Semin Dermatol 1995;14:135-9.

13. Sussman GL, Yang WH, Steinberg S. Melkersson-Rosenthal syndrome: clinical, pathologic and therapeutic considerations. Ann Allergy 1992;69:187-94.

14. Hornstein OP. Melkersson-Rosenthal syndrome-a challenge for dermatologists to participate in the field or oral medicine. J Dermatol 1997;24:281-96.

15. Donoso A, Esgueq A. Síndrome de Melkersson-Rosenthal: presentación de tres casos. Rev Med Chil 1995;123:1514-9.


Dr. Ruza

Pediatric Department

University Clinic of Navarra

P.O. Box 4209

31080 Pamplona (Spain)

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