2004
DOI: 10.1111/j.1468-3083.2004.01084.x
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Persistent unilateral orbital and eyelid oedema as a manifestation of Melkersson–Rosenthal syndrome

Abstract: Melkersson-Rosenthal syndrome (MRS) is a complex neuromucocutaneous disorder characterized by localized orofacial oedema and cranial nerve dysfunction, frequently associated with minor signs, including furrowed tongue. Complete forms are rare whereas mono- and oligosymptomatic variants are more common. A 71-year-old man presented with a 2-year history of relapsing and progressively persistent oedema of the right eyelids and periorbital region. A fissured tongue and telangiectatic rosacea had been present since… Show more

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Cited by 25 publications
(23 citation statements)
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“…598,600 The concomitance of granulomatous cheilitis and vulvitis is extremely rare. 605 The etiology is unknown but the syndrome has been considered to be a manifestation of sarcoidosis, 606 a reaction to infection or to foreign material, such as silicates, gold, and mercury, 607 and a delayed hypersensitivity to cow's milk protein or food additives. 593 The initial facial swelling may last only a few days and be soft and fluctuant, but it becomes firmer and persistent with time.…”
Section: Melkersson-rosenthal Syndromementioning
confidence: 99%
“…598,600 The concomitance of granulomatous cheilitis and vulvitis is extremely rare. 605 The etiology is unknown but the syndrome has been considered to be a manifestation of sarcoidosis, 606 a reaction to infection or to foreign material, such as silicates, gold, and mercury, 607 and a delayed hypersensitivity to cow's milk protein or food additives. 593 The initial facial swelling may last only a few days and be soft and fluctuant, but it becomes firmer and persistent with time.…”
Section: Melkersson-rosenthal Syndromementioning
confidence: 99%
“…There are a number of other even rarer inflammatory disorders, such as cold urticaria [49], relapsing polychondritis [50], lymphedematous rosacea [51,52], allergic eosinophilic gastroenterocolitis [53], Melkerson-Rosenthal syndrome [54,55], polyarteritis nodosa [56], cutis laxa [57], scleredema adultorum of Buschke [58], dermatographism [59], and tumor necrosis factor receptor-associated periodic syndrome [60]. These entities should be considered when the more common causes of periorbital edema are ruled out.…”
Section: Key Pointsmentioning
confidence: 99%
“…Idiopathic orbital inflammation [78,79] Lupus erythematosus [41,43,47,48] Cold urticaria [49] Melkersson-Rosenthal syndrome [54,55,80,81] Scleredema adultorum of Buschke [58] Allergic eosinophilic gastroenteritis [53] Relapsing polychondritis [50] Orofacial granulomatosis [82] Lymphoma [83] Polyarteritis nodosa [56] Kaposi's sarcoma [84] Protein-losing enteropathy [85] Superior vena cava syndrome [86] Type B Niemann-Pick disease [87] Esthesioneuroblastoma [88] disorder causing periorbital edema in an ophthalmology setting, we performed a retrospective case series at the University of Iowa Hospitals and Clinics Oculoplastics clinic from 2009-2011. Forty patients were referred with the chief complaint of periorbital edema.…”
Section: Retrospective Case Reviewmentioning
confidence: 99%
“…Es werden auch rezidivierende oder persistierende Schwellungen der oralen Mukosa, im Gesichtsbereich und außerhalb des Kopfes sowie verschiedene motorische und sensorische Störungen der Hirnnerven dem MRS zugeordnet [2]. Die klassische Symptomentrias wird selten beobachtet, sodass mono-oder oligosymptomatische Formen dominieren [4]. In diesem Zusammenhang müssen auch die im vorliegenden Fall anamnestische Trigeminusneuralgie und die verminderte Tränendrüsensekretion als Minorsymptome gewertet werden [1,3].…”
Section: Diskussionunclassified
“…In diesem Zusammenhang müssen auch die im vorliegenden Fall anamnestische Trigeminusneuralgie und die verminderte Tränendrüsensekretion als Minorsymptome gewertet werden [1,3]. B. orofaziales Ödem und/oder Fazialisparese) und dem histologischen Nachweis eines nicht-verkäsenden Epitheloidzellgranuloms [4]. Der histopathologische Befund zeigt perivaskuläre, nicht-verkäsende, epitheloidzellige Granulome, vereinzelt wurden auch, wie im vorliegenden Fall, endolymphatische Granulome beobachtet [5].…”
Section: Diskussionunclassified