Melkersson-Rosenthal syndrome.

The patient’s imaging features, especially the honeycomb pattern of ossific changes in the geniculate fossa, were virtually pathognomonic for ossifying hemangioma of the facial nerve.

Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous syndrome marked by the triad of recurrent nonpitting orofacial edema, fissured dorsal tongue (lingua plicata), and lower motoneuron facial paralysis. Large case series including treatment are limited. A retrospective records review was performed for the diagnoses Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis, confirmed by noncaseating granulomas on biopsy, at the Mayo Clinic in Rochester, Minnesota (1979-2009). There were 72 patients [51 women (71 %), mean age at presentation 39 years (range 8-79)] identified with facial edema with noncaseating granulomas on skin biopsy. Lingua plicata occurred in 34 cases (47 %, 95 % confidence interval 35.3-59.3 %). Unilateral or partial facial nerve palsy occurred in 14 cases (19.4, 95 % confidence interval 11.4-30.8 %). Comorbidities among those with facial edema included periodontal disease (n = 10, 14 %), history of allergic disease (n = 10, 14 %), Crohn's Disease (n = 6, 8 %), migraine headaches (n = 5, 7 %), and systemic lupus erythematosus (n = 2, 3 %). There were no patients who had low C1q or C4 levels among those who were tested. Overall, the full triad canonical of Melkersson-Rosenthal syndrome was observed in nine patients (seven female, median age at symptomatic presentation 35 years (range 10-74 years), 13 %, (95 % confidence interval 6.2-22.9 %) with a median time from first symptoms to diagnosis of 4 years (range 1-35). The medication treatments attempted in the nine patients with the full triad of symptoms included non-steroidal anti-inflammatory drugs, oral and intra-lesional steroids, metronidazole, dapsone, acyclovir, methotrexate, and thalidomide with no consistent treatment responses. The Melkersson-Rosenthal syndrome may present over the course of most of the lifespan and may require several years of observation to be diagnosed. Neurologists who observe a combination of facial edema and facial palsy in a patient should consider the diagnosis of MRS and proceed to a diagnostic skin biopsy and a trial of steroid treatment for their patient.

Melkersson Rosenthal Syndrome (MRS) is a rare neurological disorder characterized by swelling of the face, particularly one or both lips (granulomatous cheilitis), facial muscle weakness (palsy) and a fissured tongue. We present a patient with Melkersson Rosenthal Syndrome, highlighting the clinical triad of symptoms and management. A74-year-old Nigerian male presented to the Oral Medicine Clinic of the University of Benin Teaching Hospital for evaluation of right-sided facial numbness, inability to close the right eye, left facial deviation suggestive of a lower motor neuron type facial palsy of 4 days, and painless swelling of the upper and lower lips for seven days. On examination, he was found to have swelling of the upper and lower lips, multiple fissures on the tongue, right facial paresthesia, and an isolated right-sided facial nerve paralysis. He was empirically managed with 50mg prednisone daily for seven days, which was tapered over two weeks and neurobion 1 tablet daily for a month. This resulted in remission of lip swelling, however fissured tongue remained. Two months later, facial deviation had become less apparent. Patient is being followed up in the outpatient clinic. Melkersson Rosenthal Syndrome is a rare disorder with features of facial swelling, facial nerve palsy and fissured tongue. Some affected individuals may have all three of these features and others may have only one or two. The diagnosis of MRS can be made clinically when there is a complete triad of symptoms as reported in our patient.Keywords: Melkersson Rosenthal Syndrome, clinical symptoms, management

The aim of this study was to compare genetic predilection and recurrence tendency between facial palsy in Melkersson-Rosenthal syndrome (MRS) and Bell's palsy We carried out an investigation on patients with facial palsy in MRS and those with Bell's palsy who visited the outpatient department in our hospital between February 2009 and February 2013. They were asked about familial history and whether it was the first episode, with the results recorded and compared. There were 16 patients with facial palsy in MRS and 860 patients with Bell's palsy involved in the study. Familial history was positive in 5 of 16 patients (31.3%) with facial palsy in MRS and 56 of 860 patients (6.5%) with Bell's palsy (P < .01). Twelve of 16 cases (75%) with facial palsy in MRS and 88 of 860 cases (10.2%) with Bell's palsy had a history of facial palsy in the past (P < .01). Compared to Bell's palsy, facial palsy in MRS has an obvious genetic predilection and recurrence tendency.

A 28-year-old man presented with right-sided facial paralysis that had been worsening over the past eight months. He was initially diagnosed with Bell's palsy and treated with oral steroids and antiviral medication immediately after symptom onset, but experienced minimal improvement. He has a known history of conductive hearing loss in the right ear since age five due to a traumatic tympanic membrane perforation from q-tip use and subsequently underwent tympanoplasty at that time. He denied otalgia, otorrhea, vertigo, or tinnitus. Physical examination showed grade 6/6 paralysis on the right side. Audiogram from two months before presentation ago is shown in Figure 1. What is your diagnosis?Figure 1: Audiogram showing moderate-to-severe conductive hearing loss on the right side. Hearing loss, paralysis.Figure 2: Axial (horizontal) CT of the right temporal bone showing the cholesteatoma involving the middle ear, cochlea, and internal auditory canal. Hearing loss, paralysis.Figure 3: Axial (horizontal) CT of the right temporal bone showing cholesteatoma 1.2 mm above Figure 2 showing the involvement of the tympanic (middle ear) facial nerve by the cholesteatoma. Hearing loss, paralysis.Figure 4: Coronal (vertical parallel to ear) CT of the right temporal bone demonstrating that the cholesteatoma has eroded the tegmen tympani. The cholesteatoma appears to have originated medial to the malleus. Hearing loss, paralysis.Figure 5: Sagittal (vertical parallel to face) CT of the right temporal bone further highlighting the erosion of the tegmen tympani as the cholesteatoma extended medially to the cochlea and internal auditory canal. Hearing loss, paralysis.Figure 6: Sagittal (vertical parallel to face) CT Temporal bone showing the involvement of the cochlea 2 mm medial to the image in Figure 5. Hearing loss, paralysis.DIAGNOSIS: IATROGENIC CHOLESTEATOMA The most concerning aspect of this patient's presentation is the duration of his facial paralysis. Although Bell's palsy is the most frequent diagnosis for facial paralysis, the physician must begin to consider other etiologies and obtain imaging with MRI of the internal auditory canals (IACs) if the paralysis persists beyond six months. The majority of patients with facial paralysis are diagnosed with Bell's palsy, also called idiopathic facial nerve paralysis. By definition, the exact cause of Bell's palsy is unknown; however, it is believed that a large number of cases are due to edema in and around the facial nerve and is caused by the herpes simplex virus (HSV). HSV, which also causes cold sores, has been found to be present within the geniculate ganglion of affected individuals. Viral replication and reactivation within the ganglion are thought to cause edema and subsequent compression of facial nerve fibers, resulting in blockage of electrical conduction and subsequent facial paralysis. The surrounding bony architecture of the facial nerve helps to explain this phenomenon. As it courses through the labyrinthine segment of the temporal bone (the narrowest portion of the fallopian canal measuring approximately 0.68 mm), the facial nerve is completely surrounded by bone, and therefore vulnerable to compression in the event of swelling. The extent of nerve injury depends on both the degree of inflammation and how quickly treatment with steroids and antivirals can be given to reduce swelling. In cases of mild edema, there is transient compression and blockage of nerve conduction until the inflammation subsides. However, in more severe cases the nerve fibers may be crushed and rapidly degenerate. In these cases, axon regeneration usually occurs, but the new nerve fibers may not reach the intended target muscles. This results in synkinesis, in which voluntary movement in one facial muscle group causes involuntary activity of another. For example, a patient may experience involuntary blinking when trying to smile. In the case of this patient, we obtained a CT scan of the temporal bones given the history of conductive hearing loss and previous surgery (Figs. 2, 3, 4, 5). On the right side, we see bony erosion with soft tissue opacification within the petrous and mastoid temporal bone segments, including regional involvement of the labyrinthine and tympanic segments of the facial nerve, basal turn of the cochlea, vestibule, IAC, tegmen tympani, and middle ear cavity including the ossicles. Though initially treated for Bell's palsy, our patient was ultimately diagnosed with a middle ear cholesteatoma that invaded the skull base and involved the facial nerve. The diagnosis was confirmed surgically. Cholesteatomas are benign masses comprised of abnormal squamous epithelium within the temporal bone. Over time, these masses can grow large enough to cause local bony destruction with surrounding inflammation and granulation tissue. Cholesteatomas are often classified into congenital and acquired types (primary or secondary). In the primary acquired type, cholesteatomas typically arise in the setting of chronic tympanic membrane (TM) retraction. Alternatively, secondary acquired cholesteatomas occur in the setting of TM perforation with epithelial migration into the middle ear space. Given the patient's history of q-tip injury and subsequent surgery, the cholesteatoma was most likely caused by traumatic implantation of squamous epithelium or iatrogenic, i.e., caused by the surgeon not removing or implanting squamous epithelium from the middle ear. Facial nerve palsy due to cholesteatoma has been rarely reported in the literature.1 While the mechanism by which cholesteatoma causes facial nerve palsy remains unclear, several theories have been proposed. The first hypothesis is that direct compression by the cholesteatoma is responsible for causing nerve edema and subsequent ischemia. A second hypothesis is that direct contact between the cholesteatoma and facial nerve promotes an inflammatory reaction that leads to injury. This theory is supported by histological studies showing degeneration of the epineurium in facial nerve segments exposed to cholesteatoma or granulation tissue.2 A third hypothesis is that nerve injury is mediated by neurotoxic or enzymatic substances secreted by the cholesteatoma, although the significance of these factors remains controversial.3 It is important to accurately diagnose and treat cholesteatomas, as they have a strong propensity to become infected and erode through local bony structures.4 The infections and associated pathogens in cholesteatoma can be especially hard to eradicate as they are frequently polymicrobial and resistant to antibiotics. Skull base invasion of cholesteatomas carries an increased risk of deafness, facial paralysis, and intracranial complications given their location. In this patient, we see the cholesteatoma is already eroding the cochlea, creating an increased likelihood of sensorineural hearing loss in the right ear. After evaluating the extent of the disease on CT scan, surgical treatment is undertaken with the goals of removing all of the cholesteatoma and repairing damaged structures when possible. Various surgical approaches can be used, depending on the involved structures as well as surgeon comfort level. In this patient, a right middle cranial fossa or translabyrinthine approach could be undertaken. Generally, when the hearing is intact, the best approach is the middle cranial fossa. The translabyrinthine approach is reserved for non-serviceable hearing patients. If the facial nerve function does not return, the patient may receive a hypoglossal-facial jump graft. In the future, a medical device in development may allow restoration of function for the patient.5-6 BONUS ONLINE VIDEOS: VISUAL DIAGNOSIS Read this month's Clinical Consultation case, then watch the accompanying videos from Hamid R. Djalilian, MD, to review the patient's imaging for yourself. Video 1. Axial (horizontal) CT of the right temporal bone showing the extent of the cholesteatoma in the axial plane and involvement of tympanic facial nerve and geniculate ganglion. Video 2. Coronal (vertical parallel to ear) CT of the right temporal bone showing the extent of the cholesteatoma in the coronal plane and invasion of the tegmen and IAC. Video 3. Sagittal (vertical parallel to face) CT of the right temporal bone showing the extent of the cholesteatoma in the sagittal plane and invasion of the cochlea. Video 4. Axial (horizontal) CT of the left temporal bone showing the normal anatomy of the facial nerve in the axial plane. Video 5. Coronal (vertical parallel to ear) CT of the left temporal bone showing the normal anatomy of the tegmen tympani. Video 6. Sagittal (vertical parallel to face) CT of the left temporal bone showing the normal cochlear anatomy in the sagittal plane. Watch the patient videos online at thehearingjournal.com

Bell's palsy in children

Key words: Bell's palsy, brainstem glioma, magnetic reso- nance imaging Introduction Facial palsy of the peripheral type is generally seen in the pain clinic and is often treated with a stellate gang- lion block. The most common cause of peripheral facial nerve palsy is Bell's palsy, although its etiology remains controversial. The diagnosis of Bell's palsy is usually made by exclusion of other conditions such as herpes zoster oticus (Ramsay Hunt syndrome), trauma (including skull base fracture and surgery), otitis media, and neoplasm [1]. Isolated peripheral facial nerve palsy of neoplastic origin is uncommon. We herein describe a case of peripheral facial nerve palsy which was initially diag- nosed as Bell's palsy but was later found to be caused by an intrinsic brain stem tumor. Case report A 9-year-old boy presented to the Pediatric Depart- ment of our University Hospital in August 1990 with left facial weakness. His mother noticed the hyperemic conjunctiva and lacrimation of his left eye at the end of June. Consultation with the ophthalmologist revealed no abnormality in his left eye and the hyperemia im- proved with conservative therapy. In July, facial asym- metry became obvious. He was diagnosed as having Bell's palsy by a pediatrician in August and was referred to. our pain clinic. Address correspondence to: K. Kodama Received for publication on May 31, 1993; accepted on January 6, 1994 Upon examination, the patient had a left facial nerve palsy of the peripheral type (score of the facial paresis was 24/40), however, no other neurological deficits were seen. An audiogram failed to reveal a hearing abnormality. Although repeated stellate ganglion block was given, his facial palsy progressed slowly over a 2- month period, suggesting an etiology other than Bell's palsy. Magnetic resonance imaging (MRI) in September demonstrated a tumor in the left pons and brachium pontis extending into the left cerebellopontine angle (Fig. 1). The lesion was seen as a hypointense and hyperintense area on T1- and T2-weighted images, re- spectively. He was admitted to the Neurosurgical De- partment on September 29. The positive neurological findings on admission were Bruns' nystagmus, absence of left corneal reflex, decreased gag reflex, and mild trunkal ataxia, in addition to left facial nerve palsy. He underwent a wide suboccipital decompressive craniec- tomy, and biopsy of the tumor indicated low-grade glioma. In spite of postoperative radiation (60 Gy) and chemotherapy including Ranimustine and tumor necro- sis factor, he died due to tumor progression 17 months from the time of his initial symptom. Discussion Eighty percent of peripheral facial nerve palsy cases represent idiopathic or Bell's palsy, of which approxi- mately 20% can be demonstrated to have a specific etiology [2]. Peripheral facial nerve palsy with neoplas- tic origin is uncommon, and is estimated to be the cause in approximately 5% of all cases [3]. The diagnosis of Bell's palsy is unjustified unless an accurate history is taken along with a careful examina- tion of the ear and central nervous system (CNS). The differential diagnosis of neoplastic facial palsy is vast

Orofacial manifestations of Melkersson-Rosenthal syndrome: A study of 42 patients and review of 220 cases from the literature

Facial Nerve Palsy: Evaluation by Contrast-enhanced MR Imaging

Bell's palsy is the most common cause of peripheral facial nerve paralysis. Although herpes simplex virus type 1 (HSV-1) infection has been strongly suggested as a cause of Bell's palsy, the pathomechanism of the facial nerve paralysis is unclear. Previously, we had succeeded in producing an animal model of acute, transient and homolateral facial paralysis by inoculating HSV-1 into the auricle, simulating Bell's palsy. To clarify the mechanism of this facial nerve paralysis, electrophysiological testing of the trigemino-facial reflex (blink reflex) and electroneuronography (ENoG) were carried out, and a histopathological study of the facial nerve was subsequently performed. The blink reflex and ENoG were examined twice ; during facial paralysis on day 10 and after recovery on day 17. The R1 latency of the blink reflex was prolonged or disappeared on the paralyzed side during facial paralysis, but recovered in all animals with the corresponding recovery of facial nerve paralysis. ENoG values were inconsistent during facial nerve paralysis and did not normalize even after complete recovery of the facial nerve paralysis. The histopathological studies demonstrated that mixed nerve damage, demyelination and axonotomesis, were present, although demyelination was dominant. Collectively, the electrophysiological and histopathological findings suggested that the pathomechanism of facial nerve paralysis caused by HSV-1 infection is mainly due to demyelination of the nerve, which was represented as a conduction block in electrophysiological testing. The present study suggested that the facial nerve damage underlying Bell's palsy involves mixed nerve damage including demyelination and axonotomesis, and the prognosis of facial nerve paralysis is dependent on the balance of these two kinds of nerve damage.

Purpose of reviewWe aim to illustrate the potential viability of MCTD as anunderlying aetiology of Melkersson–Rosenthal syndrome. The case is probably the first description available in the literature of the Melkersson–Rosenthal as an early manifestation of mixed connective tissue disease.Recent findingsThe Melkersson–Rosenthal syndrome consists of a triad of recurrent lip and/or face swelling, fissured tongue, and intermittent facial palsy. Mixed connective tissue disease is a multisystemic disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by a high titer of antibodies to ribonucleoprotein. The paper presents a case report of Melkersson–Rosenthal syndrome with an onset in childhood that derived from vasculitis that turned out to be an early manifestation of mixed connective tissue disease. We used MRI to evaluate patient’s brain structure and Immunoblot Ena Profil 1 test to test serum autoantibodies level. The patient has a typical for Melkersson–Rosenthal syndrome triad of symptoms: bilateral facial nerve palsy, lingua plicata and facial oedema. Both TC and MRI of the head show no changes as well as laboratory tests except Anti-SS-A (Anti-Ro) and Anti-RNP autoantibody serum level that was highly positive.SummaryNeurological involvement of the MCTD usually includes, according to the frequency of the occurrence, trigeminal neuralgia, headaches, sensorineural hearing, cerebral haemorrhage, transverse myelitis, cauda equina syndrome, retinal vasculitis, progressive multifocal encephalopathy, and demyelinating neuropathy. For clinical practice it is important to remember that Melkersson–Rosenthal syndrome can also be the neurological manifestation of MCTD, especially when accompanied by other systemic symptoms.

Article1 August 1942THE OCCURRENCE OF PERIPHERAL FACIAL PARALYSIS IN HYPERTENSIVE VASCULAR DISEASEHAROLD R. MERWARTH, M.D., F.A.C.P.HAROLD R. MERWARTH, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-17-2-298 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptAlthough paralysis of the peripheral portion of the facial nerve is a very common disorder, its occurrence as a result of localized compressive bleeding in hypertensive vascular disease has been regarded as rare. Whereas hemorrhage into the facial aqueduct as a cause of facial palsy was recognized by earlier observers, their pathological findings and opinions have been ignored recently in the tabulated causal classifications of facial paralysis.Since 1925, 468 cases of facial paralysis peripheral in location have been observed. Eighteen of this number were myoclonic facial palsies. Although the ultimate picture of myoclonic paralysis resembles that of the contractured...Bibliography1. MOXON : Transactions of the Pathologic Society of London, 1869, xx, 420. Google Scholar2. GOWERS WR: Diseases of the nervous system (Am. Edition), 1888, Blakiston & Son, Philadelphia, p. 648. Google Scholar3. OPPENHEIM H: Textbook of nervous diseases, 1910, Darien Press, Edinburgh, i, p. 482. Google Scholar4. KEITHWAGENERKERNOHAN NHPJW: The syndrome of malignant hypertension, Arch. Int. Med., 1928, xli, 141. CrossrefGoogle Scholar5. AMBERG S: Hypertension in the young, Am. Jr. Dis. Child., 1929, xxxvii, 335. Google Scholar6. MAY E: Néphrite chronique et paralysie faciale, Bull. et mém. Soc. méd. d. hôp. de Paris, 1930, liv, 915-917. Google Scholar7. MONIER-VINARDPUECH P: Néphrite chronique et paralysie faciale, Bull. et mém. Soc. méd. d. hôp. de Paris, 1930, liv, 977-980. Google Scholar8. GRIFFITH JQ: Involvement of the facial nerve in malignant hypertension, Arch. Neurol. and Psychiat., 1923, xxix, 1194. Google Scholar9. GALLAVARDIN ML: Hypertension artérielle et paralysie faciale périphérique, Médecine, 1936, xvii, 186-190. Google Scholar10. MERWARTH HR: The recurrence of facial paralysis, Am. Jr. Med. Sci., 1935, clxxxix, 2, 270. CrossrefGoogle Scholar11. POLITZER A: Diseases of the ears, 1926, Lea and Febiger, Philadelphia, p. 37. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: HAROLD R. MERWARTH, M.D., F.A.C.P.Affiliations: Brooklyn, New York*Read before the Brooklyn Neurological Society January 28, 1941. Received for publication August 8, 1941. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe Use of Phototherapy for Bell’s PalsyBell's palsy: an overviewAnatomical consideration of the temporal bone as a pathogenesis of Bell’s palsyPeripheral facial palsy after varicella. Report of two cases and review of the literatureCorrelates of degree of nerve involvement in early Bell's palsyBell's PalsyIdiopathic facial (Bell's) palsy: a clinical survey of prognostic factorsBell's palsy: factors affecting the prognosis in 200 patients with reference to hypertension and diabetes mellitus‘Bell's palsy’ in accelerated hypertensionIdiopathic Facial Paralysis, Pregnancy, and the Menstrual CycleIdiopathic Facial Palsy and PregnancyMelkersson-Rosenthal syndromeTransitory unilateral facial parallysis (Bell's palsy)Tratamiento de la parálisis facialXCVI The Otological Concept of Bell's Palsy and its TreatmentSymposium: The Treatment of Facial ParalysisLXIV The Present Position of Facial Nerve Surgery 1 August 1942Volume 17, Issue 2 Page: 298-307 Keywords Facial nerve Hemorrhage Paralysis Vascular diseases ePublished: 1 December 2008 Issue Published: 1 August 1942 PDF downloadLoading ...

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

To report the causes and clinical evaluation of children with facial nerve palsy (FNP) admitted to an affiliated university hospital during a 5-year period (2011-2015). A total of 124 children were retrospectively categorised into two groups: idiopathic Bell's palsy (109 patients) and the second group into other FNP aetiologies (15 patients). All children received a standardised work-up and follow-up. Therapy consisted of steroid administration associated with antiviral treatment when a viral infection was suspected. All children of the first group had a full recovery under oral steroids within 2 months of treatment. From the second group, seven children (46%) had a viral infection based on serological findings, two of them were positive for neurotropic herpes viruses, and one had Ramsay Hunt syndrome; six children with infectious FNP had recurrent FNP on the ipsilateral or contralateral side. Five patients had FNP as a complication of acute otitis media; three of them (60%) had partial or full recovery postoperatively. One child developed FNP following temporal bone trauma that had an uneventful recovery with conservative treatment. One child suffered from Melkersson-Rosenthal syndrome, and another child presented with FNP associated with unilateral hemiparesis following an ischaemic cerebral infarct. Facial palsy in children is a manifestation of a heterogeneous group of causes. The most common aetiology of FNP in children in our study was idiopathic (Bell's palsy), followed by infective causes, such as acute otitis media and neurotropic herpes viruses. Therefore, treatment should be adapted to each patient depending on the underlying disease and severity of FNP.

The Case Presentation can be found on page 1758 DISCUSSION Analysis of the patient’s upper lip biopsy revealed the presence of well-defined, non-caseating granulomas, and oedema on the lamina propria. The findings of a granulomatous lip inflammation leading to macrocheilia, associated with plicated tongue, as in the case herein presented, are features suggestive of Melkersson–Rosenthal syndrome (MRS). Even more, the pathological aspect of the lip lesion discarded the possibility of contact dermatitis in this case, so patch test was not considered necessary. This entity is an infrequent idiopathic inflammatory disorder characterized by the classical triad of recurrent or persistent orofacial swelling, plicated tongue and facial nerve palsy (FNP) (1,2). It primarily affects young adults at the end of the second decade of life, but it is an extremely rare disease in childhood with approximately 35 cases reported in the literature (3–5). Manifestations of MRS at infancy, as well as occurrences in adults, are reported to be variable as some present with the full clinical triad of the syndrome, while others are only monoor olygosymptomatic. Cheilitis granulomatosa (also named Miescher’s chronic granulomatous cheilitis) clinically consists of a painless enlargement mainly of the upper lip, with the presence of non-necrotizing granulomas as well as oedema and perivascular lymphocytic infiltration on histopathology (6). It has been generally regarded as an entity within the spectrum of the signs of orofacial granulomatosis, and it may represent a monosympomatic or olygosymptomatic form of MRS. This finding is one of the first and most common manifestations of MRS, occurring in approximately 75% of patients (5). Its development in children is a rarity (4), and its course does not differ from that in adults (4). Plicated tongue is reported to occur only in 30% of MRS in childhood. The presence or absence of this sign is not significant for the establishment of the diagnosis because according to the review of Ziem et al., (4) it is not present in the initial phases of the disease in most children, but it can develop during the posterior course. FNP, of the peripheral type, occurs in about 30% of the MRS cases. It may be unior bilateral, complete or incomplete and frequently has a recurrent course (4,7). Facial palsy may precede the attacks of oedema by several months or years or starts simultaneously, but it more commonly develops later. The incidence and course of childhood FNP in MRS does not differ from adults. Although it constitutes the most recognized neurological sign of MRS, its occurrence is not obligatory, as in the case of our patient (7). As incomplete expression of MRS is common, full confirmation of the diagnosis requires clinicopathological correlation. Common microscopic findings include oedema, non-caseating granulomas, lymphangiectasia, perivascular mononuclear infiltration of variable intensity and fibrosis (2,8,9). In the case examined herein, the classical aspect of non-caseating granuloma was observed. In some areas of the specimens analysed, a marked oedema and lymphangiectasia were also evident. A detailed medical history is also important in the establishment of MRS diagnosis, as cheilitis granulomatosa is a finding occasionally associated with tuberculosis, leprosy and mainly with sarcoidosis and Crohn’s disease (10). The possibility of sarcoidosis and Crohn’s disease was considered highly unlikely in our patient as no gastrointestinal or respiratory complaints were reported and medical history was uneventful. Fungal and Acta Paediatrica ISSN 0803–5253