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Melkersson-Rosenthal syndrome revisited as a misdiagnosed

diseaseOzan Bagis Ozgursoy, MD, Selmin Karatayli Ozgursoy, MD, Ozden Tulunay,MD,

Ozgur Kemal, MD, Aynur Akyol, MD, Gursel Dursun, MD

Ankara University Faculty of MedicineReceived 22 November 2007

Melkersson-Rosenthal syndrome (MRS) is a rare, noncaseating

granulomatous disease that is characterized by a

triad of facial paralysis, orofacial edema, and lingua plicata

(scrotal tongue, or fissured or furrowed tongue). The classic

triad of MRS is not frequently seen in its complete form,

whereas oligosymptomatic forms are more common [1-4].

The etiology and the mechanism of the disease are still

not known; however, infectious diseases, genetic causes,

allergy, and benign lymphogranulomatosis have been

implicated as possible causes of the syndrome [5-7].

Characteristic histopathological features of MRS are

lymphoedema, noncaseating epitheloid cell granulomas,multinucleated Langhans-type giant cells, perivascular

mononuclear inflammatory infiltration, and fibrosis [7].

Identification of these features can be difficult, but their

absence does not exclude the diagnosis [8,9].

Melkersson-Rosenthal syndrome is a rare disorder that is

seen more commonly in Europe than America, yet it is

unclear whether this is an artifact [2,13,14]. The etiology of

MRS is still unknown, although genetic factors, infectious

agents, allergic reactions to various foods and food additives,

and autoimmune diseases have all been considered in theetiology of MRS [2-4,10,11,15-17]. Several reports of

familial occurrence have supported the theory of genetic

origin [5,18,19].The diagnosis of MRS is often difficult because the

presence of the complete triad of symptoms is reported in 8%

to 18% of patients [1,4]. Patients may exhibit all components

of the triad simultaneously or at different times. In a patient

with persistent or recurrent orofacial edema, the presence of

at least one of the findings of idiopathic facial paralysis or

lingua plicata is sufficient to make definitive diagnosis of

MRS [2,4,10,11]. The most dominant manifestation of MRS

is facial edema, which is acute, diffuse, painless, nonpitting,

and mostly confined to the lips [1,3,4,10,15]. The upper lip is

affected more frequently, and the edema tends to be recurrent

and lasts from hours to several weeks [7,20]. It usually

mimics angioedema; however, it is more persistent than

angioedema, and it does not respond to antihistamines andcan lead to fibrosis of the involved tissues [21]. The classic

triad of MRS was present in the first two of our patients;

however, in the second patient, the first 5 attacks were with a

complete triad, but the last attack was without facial

paralysis. In all attacks, they have orofacial edema, which

gradually recovered after treatment.Facial paralysis associated with MRS may occur months

to years before or after the onset of facial edema [20].

Relapses of PFP are frequent but eventually recover in most

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patients, as in our 2 patients. Peripheric facial paralysis may

be unilateral or bilateral, and it may be partial or complete.

Our patients presented complete paralysis in all PFP attacks.

One of them had bilateral but not simultaneous PFP.

Lingua plicata has been accepted to be congenital in 30%

to 80% of reported patients [21]. This anomaly is present in

only 0.5% to 5% of the general population, and it is

considered as a developmental malformation [1-4,10-12,15].All of our patients had fissured tongue, but their familyhistories were noncontributory.

Temporal MRI revealed no pathological finding in our

patients with recurrent PFP, and chest x-ray was normal in all

patients. Although there is no specific radiological finding

for MRS, chest x-ray and temporal and cranial computed

tomography or MRI can be used to exclude other diseases.

The histopathological investigation of the swollen lip or

facial tissues reveal the characteristic findings, including

edema, noncaseating epitheloid cell granulomas, multinucleated

Langhans-type giant cells, perivascular mononuclear

inflammatory infiltration, and fibrosis [7]. BecauseMRS is a clinical syndrome, histological evidence is not

necessary [8,9]. However, biopsy may help to diagnose MRS

and to exclude Crohn disease and sarcoidosis [21], as it did

in two of our patients.

Melkersson-Rosenthal syndrome is associated with some

other findings, including trigeminal neuralgia, paresthesias,

ocular palsies, blepharospasm, epiphora, keratitis, psychotic

episodes, and migraine [2,6,10,11,15]. In the differential

diagnosis of MRS, Crohn disease, sarcoidosis, granulomatous

blepharitis, cheilitis, contact dermatitis, facial trauma,

and Bell palsy are considered [4,11,17,22-24]. Two of ourpatients had severe diarrhea in all of their attacks. However,

no pathological finding was found on colonoscopy, and

biopsies from colon and oral mucosa lacked any ulceration

or cobble stoning. The same patients also experienced

burning sensation and discharge on their eyes. The

consultant ophthalmologist noted uveal ectropion and patchy

atrophy in iris stroma for our second patient and conjunctivitis

for the third one. Sarcoidosis is not considered in any of

our patients regarding the lack of pulmonary symptoms,

normal x-rays, and nondiagnostic consultation notes.

The modality of treatment in MRS is another issue of

controversy. Facial edema may temporarily be regressed bysystemic and intralesional corticosteroids. Antihistamines,

antibiotics, salazosulfapyridine, and irradiation have been

reported to be not useful for facial edema [25,26]. Initial

choice of treatment in facial nerve paralysis should be

medical treatment [1,2,6,10,11]. However, decompression of

facial nerve throughout its bony canal may be indicated for

patients with recurrent or persistent attacks of facial paralysis

despite medical treatment [27]. Furthermore, in severecosmetic complaints associated with persistent excess tissue

or the appearance of upper lip, reconstructive surgery can be

the treatment of choice [20]. Corticosteroid therapy was theinitial treatment for our patients. In addition, facial

decompression was performed for our first patient, and

intralesional steroid injection was applied into the edematous

lips of the last 2 patients. Respecting the patients' data and

the literature on the subject, we got the impact that MRS

patients are not appropriate candidates for facial decompression.

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Hence, we consider that corticosteroid therapy, either

systemic or intralesional, might be the current and

noninvasive treatment of choice for MRS.

In the daily practice of an otolaryngologist, it is not usual

to diagnose a patient as having MRS. Although 2 of our

patients have had the symptoms since adolescence and they

have been treated by different physicians, they could be

diagnosed as having MRS 9 and 16 years after the onset ofsymptoms. Much more interpretation of the outcome fromsuch a small number of patients would not be expressive, but

we consider that this is partly because of misdiagnosis. We

recommend that when a patient with recurrent facial

paralysis is encountered, he/she should be examined and

questioned for recurrent or persistent orofacial edema. Also,

the reverse should be considered for a patient with recurrent

orofacial edema. Lingua plicata should be searched in both

situations. Then, when MRS is diagnosed, we recommend

consultations to dermatology, immunology, gastroenterology,

and ophthalmology to rule out other diseases with

similar symptoms and to figure out accompanying problems.Lastly, these patients should be followed up regularly

because of the chronic progressive course of MRS.

We searched MRS in PubMed and found 140 articles

published between 1997 and 2007. However, only 7 (5%) of

them were published in otolaryngology journals. We therefore

believe that this study will supply an additional aspect to

otolaryngologists, in the scope of recurrent facial paralysis

and orofacial edema.

J Cutan Pathol 2009doi: 10.1111/j.1600-0560.2009.01446.x

John Wiley & Sons. Printed in Singapore

Copyrightk2009 John Wiley & Sons A/S

Melkersson Rosenthal

syndrome:ahistopathologicmysteryanddermatologicchallengeMelkersson Rosenthal syndrome (MRS) is rare disease of unknown

etiology characterized by orofacial edema, facial nerve palsy andfissured tongue. Microscopically, it shows epithelioid non-caseousgranulomas; however, edema and perivascular lymphocytic infiltrates

have been described. Two different clinical forms of MRS arepresented in this report. In the complete form (Case 1), the main

histopathologic finding was a non-necrotizing granulomatous

inflammation with 56% of the total number of cells composed of B cells(CD20+) principally located in the granulomas center and 33% beingT cells predominating in the surrounding area, of which 48% were

CD4+ and 16% were CD8+ lymphocytes. In the monosymptomatic

form (Case 2), the inflammatory cells were dispersed into the connectivetissue without granulomatous formation. B cells were scanty, and 78%

of the cells were CD45+ T cells, with 46% and 34%, CD8+ and CD4+

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phenotype, respectively. These cases showed different clinical,

histopathological and immunohistochemical forms of MRS, suggesting

different host immune responses.Kaminagakura E, Jorge J Jr. Melkersson Rosenthal syndrome: a

histopathologic mystery and dermatologic challenge.

J Cutan Pathol 2009. k2009 John Wiley & Sons A/S.Estela Kaminagakura andJacks Jorge Jr.

The etiology of MRS remains unknown; however,

infectious factors of bacterial,4 or allergic origin,5as well as hereditary and family factors6 can be

involved. Family predisposition, with autosomal

dominant characteristics and incomplete penetrancehas been reported.6 A possible hereditary etiologyis suggested by the fact that in Case 2, a

15-year-old daughter of the patient had hadepidodes of facial palsy, each lasting a month,

always proceeded by otalgia but without labialor gingival edema, or fissured tongue. However,

facial palsy can occur because of direct facial nervegranulomatous infiltration or secondary to nerve

compression by edema.7 Authors have described

MRS as a polietiological disease that has genetic oracquired predisposition to a functional disturbance

of autonomous nervous system, associated with

granulomatous reaction from an allergic response toan undescribed circulating antigen.1,6 However, thishypothesis has been contradicted in other studies.8,9

It is very hard to correlate MRS to a specific

etiologic agent and possibly it should be classified

as autoinflammatory disorder. Autoinflammatorydisorders are characterized by recurrent episodesof inflammation in the absence of pathogens,autoantibodies or antigen specific T cells as in

Crohns, or Behcets diseases and sarcoidosis.10

Granulomatous inflammation is the main

histopathological finding in MRS; however, it isnot essential to establish a MRS diagnosis asthiscan be based upon the clinical and microscopic

characteristics.11 The tuberculoid type of granulomais composed of small epithelioid cells, surrounded

by lymphocytes, plasmocytes, macrophages and diffuse

edema within the interstitial connective tissue,

whereas the lymphonodular type shows a welldemarcated,central lymphocytic nodule marginatedby plasmocytes and macrophages in the edematous

connective tissue.1 In this study, we found granulomasof the lymphonodular plasmocyte type, without

the presence of multinucleated giant cells (Langhans

type) as described in some reports.8 The infectiouspossibilities were investigated using Ziehl-Neelsonand Periodic Acid Schiff stains, which were negative

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in both cases. Ronnblom et al.12 showed that presence

of T-lymphocytes with CD4+predominance in

MRS, can initiate a cellular immune response andcontribute to granulomatous formation. However, a

decreased CD4/CD8 ratio was reported also in apatient with the monosymptomatic form of MRS.11Facchetti et al.13 did not identify B lymphocytes in

granulomas of the monosymptomatic form; instead,

they were composed of CD68+ epithelioid cells and

T lymphocyte / receptors They observed also adecrease in the T CD4/CD8 relationship. In our

study, in the complete form of MRS, the granulomaswere composed mainly of B lymphocytes inthe center and T-lymphocytes with CD4 phenotype

predominance were found in the periphery.

In monosymptomatic form, we observed that nongranulomatousformation had a lower CD4/CD8

ratio. The discrepancies in our two cases may reflectthe heterogenicity of this pathology, suggesting different

host immune responses. An alternative possibilityis that the lymphocytic infiltrates without granulation

formation may represent early stages of MRS

and non-necrotizing granulomas, cases of longerduration.3 Epithelioid granulomas and giant cellsseem to be related to an immunologic phenomenon,

especially to a hypersensitivity type IV reaction7

such as in Case 1, despite the lack of giant cells in thegranulomas.

Treatment for MRS is difficult because of

the unknown etiology.2 Case 1 was treated withcorticosteroid intralesional injections in the lip and

had a significant improvement, although without the

complete remission which has been reported in theliterature.6 Even though some authors have reported

skeleton muscle degeneration, necrosis and cicatricial

tissue formation after corticosteroid intralesionalinjections,14 these were not observed in Case 1.The patient in Case 2 had systemic corticosteroid

treatment with good results related to her lip edema.

Therewereno significant side effects.At present, bothpatients are stable at 15 months and 4 years of follow

up, respectively. In cases resistant to conventionaltherapy, the infliximab was recommended;2,15 it isa chimeric monoclonal antibody that binds soluble

bioactive TNF- and neutralizes its proinflammatory

effects.15In summary, the complete clinical form of MRSshowed non-necrotizing granulomas with CD20+

cell predominance whereas the monosymptomaticform did not show granulomatous formation andhad a lower CD4/CD8 ratio. Collaborative studies

with other centers to increase the number of

cases will be necessary to determine whether the

monosymptomatic form is the initial phase of MRSor whether the different clinical manifestations truly

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represent different host responses.

OtolaryngologyHeadand Neck Surgery (2008) 138, 246-251

ORIGINALRESEARCHOTOLOGYANDNEUROTOLOGY

Melkersson-Rosenthal syndromeMervi Kanerva, MD, Kirsi Moilanen, MSc, Susanna Virolainen,MD, PhD,Antti Vaheri, MD, PhD, and Anne Pitkranta, MD, PhD, Helsinki,FinlandSymptoms of MRS patients treated in the two departments

varied greatly according to the specialty: FP was always

present in the patient history in depORL, while edema was

seen in depDerma patients. These findings support our hypothesis

that the existing knowledge regarding MRS is

influenced by most studies being from the departments of

dermatology and oral or plastic surgery, which treat MRS

patients troubled by edema. Most of our patients in depORL

had never visited a doctor because of edema.

This study has several shortcomings. In depORL, the

computer search yielded only MRS patients with a specific

diagnosis code. More patients might have been found underthe diagnosis of FP, but with over 300 FP diagnoses a year,

we were unable to examine most of the charts. Thus, the

actual number of MRS patients is likely greater than that

presented here. The same shortcomings apply to depDerma.

Only patients with positive histology were coded under

MRS (as is correct with monosymptomatic CG), and thus,

we missed all suspected MRS patients. The classification

may also have influenced the MRS forms found in dep-

Derma.

Two triad MRS patients in our study had acute uveitis.

To our knowledge, only one report exists of uveitis in

association with triad MRS.13 One of our two patients hadan eye operation as a predisposing factor to uveitis, but the

longstanding nature of the disease and recurrences after two

years suggest additional etiological factors. These findings

might be associated with immunologic or autoimmune disturbances,

which are considered potential etiological factors

in MRS.

MRS affects all age groups.1 In our study, the median

age at onset was 24 years, which is close to previous

findings of 25 to 40 years.3,5,14 Our female:male ratio was

2:1, in accordance with previous studies,5,14 while equalpresentation3 or male predominance6 have also been reported.

Edema was the most common initial symptom in our

study as it is in the literature. 3,5,6,14 However, MRS patientswith FP had FP as the most common initial symptom, as

also described previously.1 The most common site of edema

was the upper lip, then both lips, cheeks, and tongue, in

accordance with earlier reports.3,5,6,14

FP has most commonly been estimated to appear in 20%

to 30%3-5,14,15 of all MRS cases, but reviews with 50%16 or

90%17 existences have also been published. The triad form

is found in up to 20% of all MRS cases.3-6 In our study,combining the patients of both departments, FP was present

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in 57% and the triad form in 31% of all cases. Zimmer et

al14postulated that FP episodes in MRS are initially short,

later lasting longer or becoming permanent. That was not

the case with most of our patients, although more than half

had residual symptoms from FP. LP can be found on average

in 6% of the general population, with an increasing

prevalence with age. 10 About half of the patients have had

LP in previous studies,3,5,6,14 compared to about one third inour study.

A MRS uma entidade rara, com uma incidncia estimada em alguns estudos como algo em torno de

0.36 a 1.8:100 000 por ano. Destes, a paralisia facial era evidente entre 20 a 50% do total de casos

The rarity of the disease makes assumptions about i

difficult. Hornstein15 estimated an incidence of 80 in

100,000 per year, with MRS FP patients accounting for

25%. In a 25-year prospective study of peripheral FP etiology,

18 the incidence of MRS with FP was 0.36 in 100,000

per year, corresponding to total incidence of 0.7 to 1.8 in

100,000 per year if FP is evident in 20% to 50% of all cases.

The incidence of idiopathic peripheral FP (Bells palsy) is20 to 30 in 100,000 per year18 worldwide, and in this respect

the incidence of MRS with FP in Hornstein15 (20/100,000/

year) may be excessive. In our retrospective material, the

annual incidence of MRS with FP was 0.2 in 100,000 per

year and of all cases 0.3 in 100,000 per year. We probably

missed undiagnosed patients, and only biopsy-proven cases

of MRS from the depDerma were included.

A etiologia da MRS permanence indefinida. Sugere-se que as formas monossintomticas sejam na

verdade uma apresentao de outras patologias como a Sarcoidose ou doena de Crohn, uma vez que

granulomas no necrotizantes so comumente encontrados nos exames patolgicos. A associao de MRScom paralisia facial foi encarada tambm como uma possvel associao com uma infeco por herpes

vrus em pacientes portadores de mutao

The etiology of MRS remains unsolved. The relation of

the monosymptomatic CG form with sarcoidosis and

Crohns disease has particularly been studied because of the

common finding of non-necrotizing granulomas1,4; no definite

association exists. One patient in depDerma had had

Crohns disease for over 10 years, after which she developed

CG. One of the FP patients had ulcerative colitis. We

did not find similar cases in the literature. MRS with FPresembles Bells palsy in which herpesviruses are considered

possible etiologic factors. 19 Such an etiology is also

suggested for MRS with FP.8 MRS is sometimes associated

with herpes infectiontype blisters at the beginning of

edema.3,8,15 We searched for mutations that would predispose

the patient to HSV-1 infection, but to no avail.

Casrouge et al9 found these mutations in two otherwise

healthy children with HSV-1 encephalitis. None of ourpatients had serious infections recorded in their patient

histories.

Os criterios diagnsticos definidos por Hornstein et al em 1987 caracterizavam como forma completa

era necessrio ao menos um episdio de edema combinado com paralisia facial . Sendo assim no era

necessria a apresentao completa da trade. Estes critrios foram ajustados em 1997, sendo aceito at

hoje e considerado como sndrome completa necessrio apenas que se tenha lngua plicata com edema

recorrente com histologia tpica, enquanto presena de paralisia facial recorrente deixa de ser critrio.

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O achado histopatolgico tpico da MRS mostra um granuloma linfoepiteliide infiltrante, ou muitas

vezes apresentam clusters linfoplasmociticos e infiltrado mononuclear ao redor dos pequenos vasos. O

edema o sintoma mais prevalente, mas no necessariamente persistente. Alguns autores criticam o uso

da presena de edema como critrio diagnstico, com relato de portadores de MRS sem edema to

evidente .

Typical MRS histology shows lymphoepitheloid granulomainfiltrations, which might also be replaced by smalllymphoplasmocytic clusters or mononuclear infiltrates

surrounding small vessels.2

Edema has previously been shown to be the most

common feature in MRS, affecting almost all patients and

eventually becoming persistent.1,3,5,6 This could also be

seen in depDerma patients in our study, but not in depORL

MRS patients. All patients from depDerma with

persistent edema had typical biopsy findings. Negative

histological findings in typical clinical MRS are common,14 and repeated biopsies in persistent edema cases

are recommended.3 One of our patients had slight persistent

edema in the upper lip with unspecific previous

biopsy results. New biopsies were taken during an acute

edema episode, revealing typical MRS histology. Some

investigators have suspected the need for persistent

edema and advanced disease before granulomatous infiltrations

develop.1 Another patient had nonpersistent

edema symptoms for only a year with non-necrotizing

granulomatous infiltrations found during an acute edema

episode. These two patient findings suggest that takingbiopsies during the acute edema phase is beneficial. Most

MRS patients with FP in our study had mild edema

symptoms or edema attacks were far apart. Thus, emphasizing

the importance of edema episodes in MRS diagnostics

might underestimate the prevalence and forms of

MRS in patients treated in otorhinolaryngology departments.

As major signs of MRS, Hornstein2 included palsies of

other cranial nerves apart from the facial nerve. One triad

patient in our study had experienced sudden deafness. Additional

criteria suggested for MRS by Hornstein2 includeminor signs of unilateral or bilateral sensory, motor neuron,

or autonomous disturbances. These symptoms have been

found in 80% of the patients2,5 and have been considered as

additions to the diagnosis in incomplete, oligosymptomatic

forms of the disease.2 Of the patients answering the questionnaire

in our study, all but one reported at least one

additional symptom (Table 1). Migraine was very common

in our patients (47%), as reported previously (41%).5 In twoother MRS studies,1,16by contrast, the prevalence of migraine

did not exceed the figure of 10(-20)% in the general

population.20

Como sinais maiores da MRS, Homstein incluiu paralisias de ouitros nevos cranianos a parte da

paralisia facial/. Como sinais menores, distrbios sensitivos, motores ou autonmicos. Essessintomas chegaram a ser descritos por ate 80% dos pacientes numa srie de casos.

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CONCLUSIONThe clinical picture of MRS varied according to the department

specialty where the patient was treated. MRS apparently

could be divided into two separate forms according to

the aggressiveness of the edema. Moreover, patients with

monosymptomatic CG differed from the other MRS patients

as though it were a distinct, separate disease. Our findings

support taking tissue biopsies during acute edema episodesto reveal non-necrotizing granulomatous infiltrations. UNC-

93B1 gene mutations predisposing to HSV-1 infections

could not be found. Even though the etiology of MRS is

unknown and treatments (not discussed here) are often unsatisfactory,

it is a relief to the patient to have MRS diagnosed

and the odd separate symptoms explained. We suspect

MRS is more frequent than diagnosed in cases ofnondominating edema. More studies, especially from departments

of otorhinolaryngology, could clarify the clinical

picture and the incidence of different forms of MRS, providing

us with a better understanding of this mystery.

WhatisthemosteffectivetreatmentofcheilitisgranulomatosainMelkerssonRosenthalsyndrome?EditorThe treatment of cheilitis granulomatosa (CG) in Melkersson Rosenthal syndrome (MRS) remains a challenge because of the

unclear aetiopathogenesis. Various therapeutic methods weredescribed.1 There are no randomized, placebo-controlled trials

and therefore it is difficult to compare them. Corticosteroids arewidely used; some authors recommend systemic corticosteroids inCG therapy, while others did not observe success with systemicmethylprednisone therapy.2 In our opinion, long-term systemiccorticosteroid usage is connected with the high risk of side -effects,and so it should not be the first line of treatment. Topical triamcinoloneor clobetasol in orobase may cause atrophy; intralesionaladministration of triamcinolone remains favourable, but the

response is temporary.3 Because of this reason, other non-steroidalanti-inflammatory agents are used. A very interesting alternative isclofazimine. Sussman etal.4 showed complete remission in five ofthe 11 patients with CG following the treatment. Ratzinger etal.5observed complete or partial response in majority of patients treatedwith clofazimine. Unfortunately we have no experience with

this drug. Dapson, an antibacterial and anti-inflammatory agent issuccessfully used to treat a range of dermatological disorders, especiallythose characterized by abnormal neutrophil and eosinophilaccumulation. The efficacy of dapson in CG treatment is rarelydescribed.

We read with interest the letter by Bacci et Valente. Authorsobserved long-term remission after three intralesional administrationof triamcinolone. The presented case enlarged our knowledge

about this rare condition yet it is still unclear what the effect of thetreatment depends on. Authors propose monotherapy with traimcinolone

as sufficient in CG, but there are also described cases treatedthis way without success. 6 We agree with Bacci et Valente that

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intralesional triamcinolone injections could be the first -line therapy.However, clinical course and therapeutic response of MRS is

unpredictable. It is probably connected with a type of pathogeneticbackground. Certainly, further investigations are required.In conclusion, there is no established therapy of CG in MRS,

which is caused by heterogeneous background of disease. In our

opinion, combined therapy with intralesional triamcinolone and

dapsone is very effective and a safe treatment of CG. We supposethat dapson can heighten the mode of action of corticosteroids

and allows reduction of its dose. Combined therapy with clofazimineand traimcinolone may also be very promising.M Sobjanek,,* I Michajowski, I Z_elazny,W Medrzycka-Dabrowska,A WodarkiewiczDepartamentofDermatology, Wenerologyand Allergology, MedicalUniversityofGdansk, Poland DepartamentofNursing, Medical UniversityofGdansk, Poland DepartmentofDepartmentofMaxillofacial Surgery, Medical University ofGdansk, Poland*Correspondence:M Sobjanek. E-mail:sobjanek@wp.pl

PanminervaMed. 2008 Sep;50(3):255-7.

Hashimoto's thyroiditis in Melkersson-Rosenthal syndrome patient:

casual association or related diseases?

Scagliusi P, SistoM, Lisi S, Lazzari A, D'AmoreM.

Departmentof InternalMedicineand PublicMedicine, SectionofRheumatology, UniversityofBari, Bari, Italy.

Abstract

Melkersson-Rosenthalsyndrome (MRS) isararedisorderofunknownetiology.MRS isclassicallydefined

asatriadofrecurrentorofacialedema, relapsingparalysisofthefacialnerve, andfissuredtongue. The

authorspresentthecaseofa 52-year-oldwomanwithorofacialswellingandfacialpainattacks. The

patientreportedtosufferofhypothyroidismandlaboratoryfindingsdisclosedfreetriiodothyronine (FT(3)),

freethyroxine (FT(4)), andthyrotropin (TSH) altered. Endocrinologicalconsultledtothediagnosisof

Hashimoto'sthyroiditis. Antithyroper-oxidaseantibodies (anti-TPO) werehighlyelevatedandthyroid

functiontestshadevidencedaclinicallysignificanthypothyroidism. A linkbetweenMRS and

immunologicaldisorderssuchassarcoidosis, Crohn'sdisease, unilateralanterioruveitisandmultiple

sclerosiswasdocumented. TheliteraturedidnotreportanyassociationbetweenHashimoto'sthyroiditis

andMelkersson-Rosenthalsyndrome. Thepresenceoftheanti-TPOantibodiesinthecasereportedhere

couldsuggestapossiblecorrelationbetweenimmunologicalalterationcharacteristicofautoimmune

thyroiditisandMRS.

PMID: 18927530 [PubMed - indexedforMEDLINE]