Melkersson-Rosenthal Syndrome: A Case Study

Melkersson-Rosenthal Syndrome A Case StudyDr. Shruti BohraDr Pratik B KariyaDr Seema BargaleAbstractMelkersson-Rosenthal syndrome (MRS) classically shows a triad of oro nervus facialis glob, fissured natural language and facial paralyse, more mutually the oligosymptomatic form. The orofacial s wellheading is characterized by fissured, reddish-brown, swollen, nonpruritic brims or firm edema of the saying. The facial palsy is indistinguishable from Bells palsy. The to the lowest degree common feature is fissured tongue, although seen in one third to one half of patients nonwithstanding when present it assists in the diagnosis. The histologic finding of MRS includes non slip of paperating, sarcoidal granulomas, not invariably but their absence does not exclude the diagnosis. All these findings together provoke the c arful search for provocative causes for the reactive symptom complex of the Melkersson-Rosenthal syndrome.Key words Melkersson-Rosenthal syndrome. chelitis granulom atosa, facial palsy, fissured tongue.IntroductionMelkersson-Rosenthal syndrome is considered to be a rare syndrome. Hornstein- estimated the incidence to be 0.08%.1MRS is a non-caseating granulomatous distemper display (complete or incomplete form) a triad of facial paralysis, orofacial oedema and fissured tongue (scrotal tongue, lingua plicata, or furrowed tongue). Orofacial swelling is the close to consistent and dominant feature of the Melkersson-Rosenthal syndrome. In a recapitulation of 200 patients diagnosed with Melkersson-Rosenthal syndrome, it was the most frequent initial presenting sign.2Most commonly the lips are affected, with the swelling more or lesstimes confined only to the lips. Cheilitis granulomatosa (or granulomatous cheilitis) is achronic inflammatory granulomatous swelling of the lip, which is referred as Miescher cheilitis which is generally regarded as a monosymptomatic form of the Melkersson-Rosenthal syndrome.3 Melkersson-Rosenthal syndrome commonly presents in a monosymptomatic or oligosymptomatic form. Around 200 cases are reported in a review of Zimmer et al2, we herewith report an additional case.Case reportA 16 year old female patient reported to dental OPD with the chief complaint of generalise gum treel magnification as well as swelling in top(prenominal) and lower lip since 8-9 months. Patient was apparently alright 9 months hazard when she had an come out of the closetcome of fever which lasted for 8 days and subsided with medication taken from a local clinician. It was followed by episodes of remission exacerbation of gingival enlargement with swelling of upper lip with pus discharge and bleeding to touch since 8-9 months. Then patient was referred to the department of oral examination pathology for needful.On examination face was bilaterally asymetrical with swelling of face on right side. Both upper and lower lips were swollen (markedly swollen upper lip) (Fig 1). On palpation the swelling had normal temper ature, was not painful, and was nonpitting and rubbery in consistency. Face was flattened on left side. Mandible was deviating on right side on opening of mouth. On intraoral examination generalized gingival enlargement was noticed which was covering cervical 1/third of almost all the teeth present in the oral cavity (Fig 2). Teeth preset were incisors to flake molars in all four quadrants. The tongue had deep grooves and fissures. A long prominent central groove was evident on the dorsum of the tongue discharge in a straight line with numerous small grooves and fissures running laterally from the middle third of the tongue (Fig 3).An initial diagnosis of angioedema was made. Diphenhydramine hydrochloride, degree centigrade mg, was administered intramuscularly followed by a course of diphenhydramine hydrochloride, 50 mg orally, four times a day. After 24 hours no noticeable reducing in facial swelling was evident. Therefore, re-evaluation of the diagnosis was necessary.As a resu lt of this history, recurrent cheilitis granulomatosa consistent with Melkersson-Rosenthal syndrome (MRS) was added to the disaccordential diagnosis. A biopsy specimen of the affected lip subject field was sent for histopathologic examination, confirming the final diagnosis of MRS. On histopathological examination the mucosa consisted of parakeratinized, hyperplastic, stratified squamous epithelium overlying collagenous connective tissue. Within the connective tissue there were discrete aggregations of lymphocytes, generally with a perivascular distribution however, some bad central foci of histiocytes consistent with granulomatous lesions. The histologic findings were consistent with cheilitis granulomatosa. (Fig 4)Treatment consisted of application of lip gloss to prevent cracking of the exposed mucosa. No further treatment was deemed necessary except continuation of his regular biannual preventive recall appointments. watchwordMelkersson-Rosenthal syndrome (MRS) classically s hows a triad of orofacial swelling, fissured tongue and facial palsy. Oligosymptomatic form of this syndrome is more common one of the example is Miescher cheilitis.3Melkersson-Rosenthal syndrome whitethorn often go undiagnosed for some day as in present case.History states that Rossolino in 1901 was the inaugural to describe a particular association of oedema of the face, facial nerve palsy and scrotal tongue, the syndrome was named after Melkersson and Rosenthal.4 In 1928, Ernst Melkersson, a Swedish physician, depict a 35-year-old woman with facial edema and paralysis. Shortly after, in 1931, Curt Rosenthal, a German neurologist, expound 3 patients who had fissured or plicated tongues in addition to orofacial swelling and facial palsies and proposed a link in the midst of the triad of symptoms. Subsequently, the triad of clinical findings came to be cognise as the Melkersson-Rosenthal syndrome.Although MRS is reported as commonly order during the second to fourth decades of life, there are many published reports of MRS in children and preadolescents, Roseman et al 7 described a case of MRS in a 7-year-old girl, Cohen et al 8 described four cases of MRS in children younger than the age of 11 years, and Yuzuk et al 9 described the case of a 13-year-old girl who presented with the oligosymptomatic form orlabial edema coupled with fissured tongue. Melkersson Rosenthal syndrome does not appear to shake off an obvious predilection for either sex, althoughSome claim that it is close to more common in females. 6,7,8 No particular racial predilection has been noted in MRS.Etiology of this disease remains largely unknown. 10 In granulomatous cheilitis normal lip architecture is altered by presence oflymphoedemaand noncaseating granulomas in the lamina propria.TH1 immunocytes produceinterleukin12 and RANTES/MIP-1alpha and granulomas.Expressionof protease-activatedreceptor1 and 2 occurs in orofacial granulomatosis (OFG).HLA typingmay show HLA-A2 or HLA-A11.11Pa thogenesis of MRS was suggested by Hornstien.1 He suggested that insane regulation of the autonomic nervous system leads to excessive permeability of the facial cutaneous vessels. From this abnormal circulation, nonspecific antigens then stimulate the perivascular cells to formgranulomas. Obstruction of perivascular vessels by granuloma has been proposed as a causative factor in the swelling. 1Initially, a patient with MRS usually complains of painless facial swelling that has persisted for a long time. nurture investigation may reveal a history of previous episodes of swelling associated with facial paralysis or fissured tongue that the patient has never connected.The most common symptom of MRS is recurring orofacial swelling in which the upper lip is more often affected. opposite areas affected by orofacial swelling in MRS in battle array of frequency, are cheek, nose, eyelid, alveolar process, and chin.2,12Zimmer et al 2 reported orofacial manifestations in 42 patients with M RS who were examined at their clinic they also reviewed 220 cases reported in the literature between 1965 and 1990. They found that 82% of patients presented with labial swelling, 40% had swelling in other parts of the face, not including the lip, 24% had Bells palsy, and 59% had fissured tongue. Other areas of intraoral swelling were, in order of frequency, the gingiva ( 11 % ), buccal mucosa ( 16% ) palate (8%). and tongue (7%).The swelling of the face and lip is described as recurrent, nontender, nonpitting, and firm, but not of hard consistency. The orofacial swelling is usually sudden and, in most cases, precedes facial paralysis by weeks, months, and even years. 2, 5, 6. First episodeof edema sometimes accompanied by fever and barmy constitutional symptoms (as seen in present case e.g. headache, visual disturbance) usually subsides completely in hours or days. Recurrences can range from days to years, each recurrent episode lasts longer and is more pronounced, and swelling m ay ultimately become permanent. 2,5,6The facial paralysis associated with MRS is frequently indistinguishable from Bells palsy. The site affected by paralysis usually corresponds to the site of swelling. As in the present case with facial swelling, the paralysis is on right side. Each recurring episode is more profound and lasts longer. 6,13,14 Other neurologic presentations associated with MRS are altered taste, migraine headaches, and trigeminal neuralgia.2,5,6,13,15Fissured tongue ( eg. lingual plicata) is usually found at birth and therefore considered an hazardal finding of MRS. Nevertheless, fissured tongue is ten times more likely to be found among individuals diagnosed with MRS than in the general population. 2, 6, 15. Miyashita et al 16 described MRS in a 56-year-old woman, who was afflicted with cheilitis granulomatosaca utilize by lingual candidiasis of a fissured tongue.The edematous lesion, on histopathologic investigation, is a noncaseating epithelial cell granuloma w ith perivascular mononucleated lymphocytic infiltrate. Fibrosis of the granulomas is typical in long-term and recurrent lesions. 1,2,17 Differential diagnosis includes Crohns disease, sarcoidosis, Aschers syndrome, and allergic angioedema, leprosy, tuberculosis.1,5,15,17,18,19In Lab studies Serum angiotensin-converting enzymetestmay be performed to help exclude sarcoidosis.Reactions to metals, food additives, or other oral antigens are excluded by employ mend tests which may be associated with some cases of granulomatous. 20 If found positive they are advised to avoid the allergen. Imaging studies like gastrointestinaltractendoscopy, radiography, and biopsy may be used to differentiate from Crohn disease. Chest radiography or gallium orpositron emission tomography(PET) scanning may be found helpful in excluding sarcoidosis and tuberculosis. Orthopentamogram (OPG) helps in ruling out presence of a chronic dental abscess.A final diagnosis of MRS is made from the clinical history and histopathologic assessment of the edematous tissue, which usually includes the lip. When biopsy of the edematous tissue is difficult or not warranted, then a history of recurrent idiopathic facial swelling associated with atleast one of the following 2 entities, idiopathic facial paralysis (ie. Bells palsy) or fissured tongue, is sufficient to make a positive diagnosis of MRS.Treatment of MRS is aimed at the facial swelling and the paralysis. Because the etiology and pathogenesis of MRS are not well understood, treatment continues to be empirical and, in most cases, unsuccessful. Fortunately, both the swelling and the paralysis are selflimiting and usually go into remission on their own. Treatment with oral prednisone or nerve decompression has been shown to be effective.1,5,6 Treatment with oral prednisone or nerve decompression has been shown to be effective.5 Also, interlesional injections with triamcinolone hexacetonide suspension have been shown to provide temporary benefit.1 2 Plastic surgery may be considered in the case of permanent fibrotic lesions.Nevertheless, thorough documentation by many dentists eventually allowed MRS to be included in the differential diagnosis. This case highlights the importance of good history taking, regular follow-up and thorough documentation for proper patient management.ReferencesHornsteinOP. Melkersson-Rosenthul syndrome A neuromucocutaneus disease of complex origin. Curr ProbI Dermatoi 1973 5117-156.Zimmer WM, Rogers RS. Reeve CM, Sheridan PJ. Orofacial manifestations of MeIkersson-Rosenthal syndiume A study uf 41patients and review of 22tn cases from the literature. Oral Surg Oral Med Oral PiU ho I IW7461O-6I9.Rogers RS 3rd Melkersson-Rosenthal syndrome and orofacial granulomatosis, Dermatol Clin.1996 Apr14(2)371-9Magid El Shennawy and Galala El Enany Melkersson-Rosenthalsyndrome Review of theliteratureand report of acase, The Journal of Laryngology Otology Volume87 / Issue09 /September 1973, pp 898-902Green RM. Ro gers RS, Melkersson-Rosentlial syndrome A review of 36 patients. J Am Acad Dermatoi 1989211263-1270.Orlando MR, Atkjns JS Jr. Melkersaon-Rosenthal syndrome. plastered Otolaryngol Mead Neck Surg I99O116728-729.Roseman B, Fryns JP, Van den Bergle C Melkersson-Rosenthal syndrome in a 7-year-old girl. Pediatrics 197S61490-491.Cohen HA. Cohen Z, Ashkenasi A, Straussberg R. Frydman M, Kauschansky A, Varsano 1. Melkersson-Rosen thai syndrome. Cutis 199454327-32S.Yuzuk S, Trau H, Levy A. Shewaeh-Millet M. Melkersson- Rosenthal syndrome. Int J Dermatoi i9SS24456-457.Tilakaratne WM, Freysdottir J, contingency F. Orofacial granulomatosis review on aetiology and pathogenesis.J Oral Pathol Med. Apr 200837(4)191-5.Ketabchi S, Massi D, Ficarra G, et al. Expression of protease -activated receptor-1 and -2 in orofacial granulomatosis.Oral Dis. Jul 200713(4)419-25.Miele FA Jr. Tlie big lip. Diagnostic and treatment considerations. Gen Dent 199442O.i8-59.Graff-Badlord SB. Melkersson-Rosen thai syndr ome, A review of the literature and a case report. South Afr Med J 19816071-74,Balatiieh AB. Pillai KG, Maiisour M. Ai-Khail AA. An unusual case of the Melkersson-Rosenthal syndrome. Oral Surg Oral Med Oral Pathol 199580289-292.Winnie R, DeLuke DM. Mel kersson-Rosenthal syndrome Review of literature and case report. Int J Oral Ma.illofac Surg 199 1115-117Miyashita M, Baba S, Suzuki H. Role recurrent oral caniiidiasis associated with lingua plicata in Me I kersson-Rosen thai syndrome. BrJ Dermatol 199l.l2.ll 1-312.Minor MW Fo RW, Bukant SC, Lockey RF. Melkersson- Rosenthal sjndrome. J .Miergy Clin Immunol l9a7S064-67.Kano Y, Shiohara T. Yagita A, Nagashima M. Association between cheilitis granulomatosa and Crohns disease. J Am Acad Dermatol 1993281801.Lloyd DA, Payton KB, Guenter L, Frydman W. Melliersson-Rosenlhal syndrome and Crohns disease One disease or two? Report of a case and discussion ofthe literature. J Clin Gastroenterol I99418213-2I8.Fitzpatrick L, Healy CM, McCartan BE, Flint SR, McCreary CE, Rogers S. Patch testing for food-associated allergies in orofacial granulomatosis.J Oral Pathol Med. Jan 201140(1)10-3.Figure legendsFigure no 1) extraoral moving-picture show showing swollen upper and lower lips.Figure no 2) intraoral photograph showing generalized gingival enlargementFigure no 3) intraoral photograph showing deep grooves on dorsal heighten of tongueFigure no 4) photograph showing histological presentation of chilitis glanduralis at low power (10X) and high power (40X)Learning pontsConcomitant presence of orofacial swelling, fissured tongue and facial palsy or any one of them may be a case of Melkersson-Rosenthal syndrome (MRS).A final diagnosis of MRS is made from the clinical history and histopathologic assessment of the edematous tissue.MelkerssonRosenthal syndrome may recur intermittently and can become a chronic disorder. metallic History, Properties and Nanoscale Analysisamber History, Properties and Nanoscale AnalysisSince first extr acted in the 5th century, Gold has been regarded as one of the most important matters in the world. When divided into smaller fragment coats, way below 100nm it becomes even more precious. The visual, catalytical and electronic properties of Gold nanoparticles differ greatly from those of their muckle counterparts. This is mainly referable to the large surface area-to-volume ratio as well as the spatial confinement of the free electrons of Gold nanocyrstals.Gold at Bulk scaleNoble metals such as Gold, silver and platinum exhibit plasmonic properties. When the surface of a noble metal is hit with incident light, electrons which are situated on the surface begin to oscillate. This is known as the surface plasmonic resonance (SPR), as illustrated in figured 1.Metals are able to get and reflect light with great efficiency to their SPR. It is because of these plasmonic properties that noble metals such as Gold, silver and platinum are widely used in jewellery. Moreover being highly reflective metals makes them very appealing to the eye. Plasmonic properties arise cod to noble metals having delocalised electrons on the surface. An atom consists of protons, electrons and neutrons. The nucleus is made up of protons and neutrons and the electrons spin around the nucleus in different orbitals. There are many orbitals in metals which overlap and form metallic bonding between the atoms. In the bulk form, there are many delocalised electrons within the metal that cause metallic bonding. This in turn allows the electrons to flow freely between the nuclei. Good electrical and thermal conductivity is due to the delocalised electronsGold at NanoscaleReflection does not occur in particles which are smaller than the wavelength of the incident light however there is still an interaction between the nanoparticles and the light. The two main interactions are light scattering and light absorption. Electrons tend to oscillate at the like frequency as the light which was abso rbed cause a dipole moment around the nanoparticles where all the electrons on the surface of the nanoparticle are oscillating. The oscillating dipole is known as a localised surface plasmon resonance (LSPR). Electromagnetic shaft of light is released when these electrons are oscillated. This can be seen in figure 2. The secondary electromagnetic radiation released by the nanoparticles is called scattered light. The electromagnetic radiation that is released has the same frequencyHistory The synthesis of colloidal gold, or nanogold as it is now called, has been known to man since the ancient times. Although the process was not fully understood, synthesis of colloidal gold was crucial to the 4th century Lycurgus cup. The Lycurgus cup was known to change colour depending on the direction of light. Later it was used as a method for staining glass.A potion made from gold, which was also known as an Elixir of Life was discussed, and may also have been manufactured, in ancient times. I t was not until the 16th century that the alchemist Paracelsus, claimed that he had created a potion called Aurum Potabile. (latin potable gold).It was in the 17th century that the glass-colouring process was refined byAndreus CassiusandJohann Kunckel, allowing them to produce a deep-ruby coloured form of glass. all the same modern scientific evaluation was first made by Michael Faraday in the 1850s. Faraday is said to have been inspired by previous work done by Paracelsus. In 1857 Faraday prepared the first pure sample of colloidal gold, which he called activated gold, in 1857. Phosphorus was used to reduce a solution of Gold chloride.For a very long time chemists were unclear about the composition of the Cassius ruby-gold. Several chemists suspected Cassius ruby-gold to be a gold tin compound due to its preparation, However it was Faraday who was the first to recognize that the colour was actually due to the minute size of the gold particlesThe first colloidal gold in solution wa s first prepared in 1898 by Richard A. Zsigmondy.Shape and size tuningFine tuning of shape and size in a controlled environment is one of greatest challenges faced by material scientists. These factors are not only very important in the rational design of nanomaterials, but are also as as important for their applications. This is because many of their catalytical, optical and electronic properties of nanomaterials depend greatly on their size and shape. In Gold nanorods the longitudinal plasmon wavelength exhibits a nearly one-dimensional dependence on their fit ratio, making it one of the most intriguing properties possessed by Gold nanorods. Moreover, even when looking at Gold nanorods with the same medical prognosis ratio, the plasmon resonance properties are strongly dependent on the shape of their head.Over the past five years, the ability to finely tune the shape and size of Gold nanorods, has made huge progress.Seed mediated growthGold nanospheresChanging the diameter of the sphere can easily and effectively tune the optical properties of gold nanospheres. This can easily be shown using the Mie Theory, which has been successfully shown to model the light scattering and absorption properties of spherical particles on a nanometer scale.The unabated peak in the spectrum for each particle can be seen in figure 9. This peak is caused by the oscillating electrons which in turn produce a single dipole. Increasing the diameter of the gold nanosphere results in shifting the peak to a higher wavelength, this indicates the interaction between the light and particles is changing. In comparison to bulk gold (figure 4), the optical properties of gold nanospheres differ immensely.The local medium can also change the optical properties of the particles. This is due to the LSPR of the particle interacting with the medium. A change in the medium can result in a measurable change in the optical properties of the particles. In addition shape of the gold nanoparticles can significantly alter the optical properties.Gold nanorodsGold nanorods are of great interest due to their biocompatibility and NIR ( near infra-red) optical properties. The shape of the gold nanorod is what determines its NIR properties. The non-spherical shape of the particle causes two different dipoles to form when interacting with light. The thwartwise dipole (diameter) and the longitudinal dipole (length) account for the oscillating electrons throughout the particle. The dipole interactions affect the optical properties of the particles.GNR LSP illustrationOptical properties of GNRsThe aspect ratio (A.R) is defined as the length over the diameter of the Gold nanorod (GNR). The shape of the particle causes the absorption and light scattering spectrum to have two peaks longitudinal and transverse. The interaction between these two dipoles causes the longitudinal peak to be observed in the visible to NIR region. The longitudinal peak is much more intense than the transverse p eak and can be tuned by changing the aspect ratio of the gold nanorods. As the A.R of the gold nanorods is increased, the longitudinal peak shifts into the NIR region.The optical properties of GNRs have been successfully modelled using Gans theory and Discrete Dipole Approximation (DDA). Two equations were derived using the two theories to output the longitudinal peak wavelength by inputting a given aspect ratio.GANS THEORYDDAThe two equations can predict the A.R of GNRs using absorption data collected from the UV-Vis-NIR spectrophotometer. The equations can give preliminary estimation of the A.R before observing the particles on a TEM. Figure 11 displays the absorption data, estimated A.R from theory, and a visual representation for a set of GNRs with different aspect ratios. The tunable optical properties of the GNRs make them very worthy for a wide range of applications. The visible representation shows a colour shift as the A.R changes.Visual representation of different shapes and sizes of GNRsNormalised absorption of the particles