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 the age of 50 and 60 years, respectively. The patient was also affected by essential hypertension and diabetes mellitus. A skin biopsy showed a marked upper dermal oedema, and small epithelioid cell granulomas arranged in perivascular and perilymphatic location. Collections of small epithelioid cells were occasionally observed within lymphatic spaces. No acid-fast bacteria, fungi or foreign bodies were detected. Intralesional corticosteroids induced transient improvement, whereas minocycline, clofazimine and dapsone have been ineffective. MRS may present with unilateral eyelid and periorbital swelling. Differential diagnoses of such cases may include a variety of cutaneous, ophthalmic and systemic diseases.

The granulomatous reaction pattern

“…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.…”

Periorbital edema

“…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.…”

“…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.…”

75‐jähriger Mann mit persistierenden bilateralen Lidödemen

“…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].…”

“…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].…”