Melkersson-Rosenthal Syndrome Research Articles

Melkersson-Rosenthal Syndrome Presenting as A New Daily Persistent Headache: Relief with Dapsone

Melkersson-Rosenthal Syndrome Presenting as A New Daily Persistent Headache: Relief with Dapsone

Peripheral Facial Palsy in the Setting of Melkersson-Rosenthal Syndrome

Melkersson-Rosenthal syndrome is characterized by recurrent periph­eral facial palsy, recurrent or persistent orofacial edema and a fissured tongue. However, this clinical triad occurs only in a minority of cases; mono or oligosymptomatic forms are much more common. The au­thors describe a case report of a 39-year-old woman with Melkersson- Rosenthal syndrome, discuss the evolution of the disease and treat­ment, highlighting the need of a high clinical suspicion index and a regular follow-up to reduce progression of deficits

Alteraciones neurológicas relacionadas con la enfermedad de Crohn

The incidence of alterations of the central and/or peripheral nervous system in a patient with Crohn s disease (EC) is 33.2%. Casual association may occur in 19.3% of the cases. The disorder of the nervous system may precede the diagnosis of EC. We review the main neurological complications of EC. The predominant cerebral vascular pathology is arterial although cases of venous thrombosis have been reported. One of the mechanisms involved is the state of hypercoagulability secondary to thrombocytosis and increase in the factors V, V111 and fibrinogen. In other cases there was confirmation of the presence of antiphospholipid antibodies and lupus anticoagulant, and therefore of an autoimmune mechanisms being involved. Other neurological features include peripheral neuropathy (axonal, demyelinating and autonomic), myopathies, pseudotumor cerebri, papilloedema, psychiatric disorders (anxiety, phobias, depression) and association with syndromes such as multiple sclerosis, Cogan s syndrome, Melkersson Rosenthal syndrome, connective tissue disorders and vasculitis. As well as hypercoagulability being one of the pathogenic mechanisms of cerebral ischaemia, there is alteration of humoral and cellular immunity in patients with EC. This justifies this and other neurological manifestations, and explains its association with other immunity disorders.

Granulomatous cheilitis and Borrelia burgdorferi: polymerase chain reaction and serologic studies in a retrospective case series of 12 patients.

Granulomatous cheilitis (GC) is a chronic granulomatous inflammation of the lips of unknown etiology, which may be associated with peripheral facial nerve paralysis and/or lingua plicata (Melkersson-Rosenthal syndrome [MRS]). Borrelia burgdorferi is a spirochete that causes Lyme borreliosis, a multisystemic infectious disease with frequent occurrence of facial nerve paralysis. An etiologic role of B burgdorferi in various granulomatous diseases has been suggested. The present study was performed to examine a possible causative role of B burgdorferi for GC/MRS by B burgdorferi-specific polymerase chain reaction analysis of biopsy specimens from affected lip tissue and determination of B burgdorferi IgG and IgM serum antibodies using enzyme-linked immunosorbent assay and immunoblot tests. We examined a retrospective case series of 12 patients with GC/MRS from a Lyme borreliosis endemic area (median duration of disease, 8 months [range, 3-348 months]). Borrelia burgdorferi-specific DNA could not be amplified by polymerase chain reaction in any of the 12 patients. One (13%) of 8 patients tested had a serum B burgdorferi IgG response on enzyme-linked immunosorbent assay, and 2 patients (25%) had an IgM response, but immunoblot testing yielded negative results in all 8 patients. The results of the present study do not indicate that B burgdorferi has an etiologic role in GC/MRS.

Orofacial edema: a diagnostic and therapeutic challenge for the clinician

Background and Conclusion A case of a 41-year-old patient with a 5-year history of chronic recurrent angioedema, refractory to conservative treatment is presented. The results of the case report suggest that in differential diagnosis of angioedema, in addition to usual causes, the allergist-immunologist needs to consider Melkersson-Rosenthal syndrome, which can present with a variety of symptom-combinations of the classic triad. The distinguishing characteristics of the Melkersson-Rosenthal syndrome are its refractoriness to the usual anti-inflammatory therapy and the need to consider corrective cosmetic surgery, which may benefit some patients. A case of a 41-year-old patient with a 5-year history of chronic recurrent angioedema, refractory to conservative treatment is presented. The results of the case report suggest that in differential diagnosis of angioedema, in addition to usual causes, the allergist-immunologist needs to consider Melkersson-Rosenthal syndrome, which can present with a variety of symptom-combinations of the classic triad. The distinguishing characteristics of the Melkersson-Rosenthal syndrome are its refractoriness to the usual anti-inflammatory therapy and the need to consider corrective cosmetic surgery, which may benefit some patients.

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We report a case of Melkersson-Rosenthal syndrome. The case involved an eight-year-old girl. She had experienced left facial paralysis and its spontaneous regression two years before she first came to our clinic with left recurrent facial paralysis and lower lip swelling. Evoked electromyography revealed that the ratio between left and right was 5%. Recovery of facial nerve function was observed after administration of a steroid. Six months later, right facial paralysis occurred, but it disappeared after steroid therapy. Three years after her first visit, right facial paralysis recurred. Because lingua plicata was also exhibited, the diagnosis of Melkersson-Rosenthal syndrome was confirmed. Complete recovery from right facial paralysis was observed after steroid therapy. Although her mother had a history of sarcoidosis, the patient's chest roentgenography, serum angiotensin converting enzyme level, and ophthalmological evaluation for uveitis revealed no signs of sarcoidosis.

Melkersson-Rosenthal syndrome in childhood: Successful management with combination steroid and minocycline therapy

The Melkersson-Rosenthal syndrome consists of a triad of recurrent lip and/or face swelling, fissured tongue, and intermittent facial palsy. Onset of the symptoms may occur during childhood, and treatment of the condition is difficult. We describe two children with Melkersson-Rosenthal syndrome in whom combination treatment with prednisone and minocycline proved effective and well tolerated. (J Am Acad Dermatol 1999;41:746-8.)

Melkersson-Rosenthal syndrome treated with sulphasalazine

Introduction: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of symptoms labial/facial oedema, fissured tongue and facial palsy. The majority of patients present with an incomplete form, commonly with only labial oedema (Miescher cheilitis). Several treatments have been reported to be effective, but none has proved its efficacy in well-designed controlled trials. OBJECTIVES: As there is frequently evidence of granulomas in lip biopsies and as sulphasalazine is the drug of choice in other granulomatous disorders, such as ulcerative colitis, we proposed to conduct a prospective study of the efficacy of sulphasalazine in MRS. PATIENTS AND METHODS: We included seven MRS patients, five females and two males, with an average age of 30.71 years (SD ± 9.79 years). The average duration of disease was 10 years (SD±8.98 years). Other diseases that can appear as labial oedema were ruled out. We also performed quantitative and qualitative assessment of the enzyme gIucose-6-phosphate-dehydrogenase (G6PD), which was always normal. RESULTS: An improvement was noticed in labial oedema in five patients (71.4%) after an average of 11.4 months of treatment (2-20 months). CONCLUSIONS: Sulphasalazine seems to be an efficient treatment in MRS, although controlled studies are necessary, which also do not exist for the other proposed treatments for this syndrome.

Cutaneous and lymphadenopathic sarcoidosis: Report of a case

Granulomas of the skin may be classified in several ways. They are either infectious or non-infectious in character, and they contain areas of necrobiosis or necrosis, or not. Responsible infectious agents may be mycobacterial, fungal, treponemal, or parasitic organisms, and each case of granulomatous dermatitis should be assessed histochemically for those microbes. In the non-infectious group, examples of necrobiotic or necrotizing granulomas include granuloma annulare; necrobiosis lipoidica; rheumatoid nodule; and lupus miliaris disseminates faciei. Non-necrobiotic/necrotizing and non-infectious lesions are exemplified by sarcoidosis; foreign-body reactions; Melkersson-Rosenthal syndrome; Blau syndrome; elastolytic granuloma; lichenoid and granulomatous dermatitis; interstitial granulomatous dermatitis; cutaneous involvement by Crohn disease; granulomatous rosacea; and granulomatous pigmented purpura. Histiocytic dermatitides that do not feature granuloma formation are peculiar reactions to infection, such as cutaneous malakoplakia; leishmaniasis; histoplasmosis; lepromatous leprosy; rhinoscleroma; lymphogranuloma venereum; and granuloma inguinale.

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Melkersson-Rosenthal syndrome: Review of literature and case report of a 10-year misdiagnosis: Balevi B. Oral Pathol 28:265, 1997

Melkersson-Rosenthal syndrome is classically described as a triad of orofacial swelling, facial palsy, and fissured tongue. More often this syndrome presents in its oligosymptomatic forms. Melkersson-Rosenthal syndrome may not be as rare as suspected but rather a syndrome that often goes undiagnosed. Presented is the case of a mentally challenged man who was eventually diagnosed with Melkersson-Rosenthal syndrome only after being misdiagnosed and incorrectly treated for an odontogenic infection for more than a decade.

Idiopathic Granulomatous Inflammation of the Female Genital Tract

A 35-year-old woman had recurrent, histologically confirmed idiopathic granulomatous inflammatory lesions involving the vulva, anoperineum, vagina, and cervix. This is the first report of involvement of the entire lower female genital tract in this pathological process. The disease has clinical and histological characteristics similar to the genital manifestations of Crohn's disease and the MelkerssonRosenthal syndrome (cheilitis granulomatosa of Miescher). In some cases, vulvitis granulomatosa has a temporal relationship with both of these clinical entities. These three diseases-Crohn's disease, the Melkersson-Rossenthal syndrome, and vulvitis granulomatosa-could reflect identical granulomatous disease, but do not necessarily result from a common etiological factor. The treatment of these conditions remains surgical excision with or without intralesional corticosteroid injections.

Diseases of the lips

The vermilion of the lips was conceptualized by Jean Darier as the semi-mucosa. The anatomy of the lips is transitional from skin to mucous membrane. This article emphasizes inflammatory diseases of the lips known as cheilitis. Angular cheilitis is a reactive process with several possible causes, including infections, mechanical, nutritional deficiency, and various dermatoses. Contact cheilitis may be caused by a primary irritant or a delayed hypersensitivity allergic reaction to contactants. Plasma cell cheilitis is a reactive periorificial mucositis. Exfoliative cheilitis is also a reactive process, probably secondary to factitious activity of the patient. Cheilitis glandularis is a chronic inflammatory disorder of the labial salivary glands and their ducts. There are three forms: simple, superficial suppurative, and deep suppurative. A premalignant potential is present in cheilitis glandularis. Cheilitis granulomatosa is one manifestation of orofacial granulomatosis. The granulomatous conditions of Melkersson-Rosenthal syndrome, sarcoidosis, and Crohn's disease may be associated with cheilitis granulomatosa or it may stand alone as Miescher's cheilitis. Actinic cheilitis is another premalignant form of cheilitis that is amenable to a variety of therapeutic modalities.

Granulomatous Blepharitis as a Sign of Melkersson-Rosenthal Syndrome

The objective of our study was to describe the ophthalmic features and histologic eyelid findings of Melkersson-Rosenthal syndrome. Three patients with eyelid edema underwent eyelid biopsy to establish the diagnosis of Melkersson-Rosenthal syndrome. Of the three patients, only one patient manifest the classic triad of facial edema, facial paralysis, and furrowed tongue. Histoloppgically, the eyelid skin in all patients showed characteristic perilymphatic granulomas with marked dermal edema. The histopathologic features assist in establishing the diagnosis of Melkersson-Rosenthal syndrome in oligo- and monosymptomatic patients. Melkersson-Rosenthal syndrome should be considered in patients presenting with eyelid edema of unknown etiology and biopsy performed.

Melkersson-Rosenthal syndrome--a challenge for dermatologists to participate in the field of oral medicine.

Melkersson-Rosenthal syndrome (MRS) is a neuro-muco-cutaneous disorder involving remittently both the oro-facial innervation and muco-cutaneous tissues in a pathosis of complex origin characterized by recurrent edema, facial or other palsies, and nerval dysfunctions frequently associated with plicated tongue. Biopsies taken from the edematous tissues often reveal a temporary pattern of moderate epitheloid granulomatous inflammation scattered scarcely within remarkable tissue edema. However, this histological pattern is not a prerequisite for the diagnosis of MRS. The disease usually runs an intermittent and unpredictable course over years or decades and may have, if the edemas involve the tongue or the central nervous system, an ambiguous outcome. Greatly disfiguring oro-facial swellings often result from secondary persistence of the primarily recurrent edemas. A classification on grounds of different 'major' and 'minor signs' of MRS is proposed in the present paper. In recent years, novel therapeutic approaches involving either oral clofazimine or laser beam acupuncture (according to the principles of traditional Chinese medicine) have proven to be successful in some cases of MRS. Dermatology could play a larger role in oral medicine by taking diseases such as MRS into account in studies among specialties dealing with oro-facial pathoses.

Melkersson-Ronsenthal Syndrome

Melkersson-Rosenthal Syndrome (MRS) is a rare condition that cause and pathogenesis is not known. Triad of syndrome are recurrent orofacial edema together with cheilitis of the lip, recurrent peripheral type facia[ nerve paralysis and fissural tongue. But complete triad is seen only one third of the patients. Most frequent and diagnostic symptom of the disease is orofacial edema. Disease affects young ages. Facia! nerve paralysis likes to Bell's palsy and tends to recur. Far treatment, conservative approach with steroids is preferred. If it recurs frequently, total facia! nerve decompression is suggested. In this paper, two cases of MRS with complete triad were presented and clinical presentations and treatment of this syndrome were discussed.

Pathological findings in the facial nerve sheath in Melkersson-Rosenthal syndrome

To our knowledge, this is the first report of electron microscopic findings in the facial nerve sheath in Melkersson-Rosenthal syndrome. We performed decompression of the right facial nerve on a patient with Melkersson-Rosenthal syndrome. A part from the facial nerve sheath was obtained and observed with a transmission electron microscope. There were some fibers 180 to 200 nm in diameter which had obscure cross bandings, and also accumulations of these degenerated fibers among normal collagen fibers.

MELKERSSON-ROSENTHAL SYNDROME AND OROFACIAL GRANULOMATOSIS

The Melkersson-Rosenthal syndrome is a rare disorder of unknown etiology characterized by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. Exacerbations and recurrences are common. The orofacial swelling is characterized by fissured, reddish-brown, swollen, nonpruritic lips or firm edema of the face. The facial palsy is indistinguishable from Bell's palsy. The fissured tongue is seen in one third to one half of patients and, although the least common manifestation, its presence assists in diagnosis. The classic triad is not seen frequently in its complete form; therefore, diagnosis is difficult. This is particularly true because monosymptomatic and oligosymptomatic variants are seen more commonly. Cheilitis granulomatosa of Miescher is an example of a monosymptomatic variant of the Melkersson-Rosenthal syndrome. The histologic findings of noncaseating, sarcoidal granulomas support the diagnosis. These granulomas are not invariably present, and their absence does not exclude the diagnosis of the Melkersson-Rosenthal syndrome. Thus, the Melkersson-Rosenthal syndrome is a disease with elements of orofacial granulomatosis. Orofacial granulomatosis is a clinicopathologic entity describing oral lesions with noncaseating granulomas. The spectrum of this entity includes patients with oral Crohn's disease, patients with oral lesions who will develop typical bowel symptoms of Crohn's disease in the ensuing months to years, patients with tooth-associated infections, patients with sarcoidosis, and patients with food or contact allergies. The value of the clinicopathologic construct of orofacial granulomatosis is to provoke the careful search for provocative causes for the reactive symptom complex of the Melkersson-Rosenthal syndrome.

Facial granulomatous lymphoedema and syringomyelia

The term orofacial granulomatosis was introduced for orofacial lesions which resemble Crohn's disease clinically and histologically in patients without gastrointestinal disease. The Melkersson-Rosenthal syndrome and its oligosymptomatic form Meischers granulomatous cheilitis are usually felt to be part of the same spectrum of disease. Oedema usually involves tissues of the lower half of the face. We describe a patient with histological changes consistent with those found in orofacial granulomatosis, but with isolated localized oedema of the right periorbital tissues. The patient also had syringomyelia which has not previously been described in association with orofacial granulomatosis.

An unusual case of the Melkersson-Rosanthal syndrome: A case report

A case of Melkersson-Rosenthal syndrome in a woman with a history of trauma and infection is presented. In addition to the classical triad of signs, the patient also exhibited several other features of rare occurrence associated with Melkersson-Rosenthal syndrome. Clinical diagnosis was confirmed by biopsy, which revealed the characteristic features of Melkersson-Rosenthal syndrome.