Melkersson-Rosenthal Syndrome

Classic triad is facial palsy, lingua plicata (fissured tongue) and orofacial edema

• Presentation with eyelid edema is more recently recognized (Lin, 2016).
• Patients with lid edema can have normal tongue
• Facial palsy can be incomplete (Lin, 2016).

Etiology

• Unknown etiology
• Association with parvovirus b19 viremia has been proposed (Demaria, 2009).

Epidemiology

• Rare condition
• No gender or racial predilection.
• In a review of 21 patients managed in a facial nerve center the average age at presentation was 44 years (22-67) (Rivera-Serrano, 2014).
  • 2/3 were female

History

• Orofacial swelling is the most common symptom.
• Only 25% of patients have classic triad.
• Mucocutaneous granulomatous inflammation.
• Upper lip swelling most common.
• Periorbital, cheek, and chin may be affected.
• Repeated episodes of swelling at irregular intervals.
• Facial palsy can be recurrent or persistent.
• Most commonly presents with unilateral facial weakness, progresses to bilateral in 2/3 of patients.

Clinical features

• Can present as unilateral eyelid edema (Reddy, 2017).
• Can present with conjunctival edema (Heinz, 2001).
• Bilateral eyelid edema most common ophthalmic presentation.
• Although fissured tongue is the most recognizable feature, it is least common.
• May have a family history (Liu, 2013).
• Can have elevated protein in cerebrospinal fluid (Liu, 2013).
• Granulomatous blepharitis can manifest as long term firm, nonpitting persistent eyelid edema for 1-3 years (Yeatts, 1997).

Laboratory testing and radiologic procedures

• Head and neck imaging can demonstrate facial soft tissue edema.
• Histopathology shows dermal edema and non-caseating granulomatous inflammation.
• Special stains for fungi and acid fast bacilli are negative.
• No foreign bodies with polarization.
• Negative systemic work-up for sarcoidosis, rosacea, Crohn’s disease and granulomatosis with polyangiitis.
• Nerve conduction studies can confirm facial nerve dysfunction.   
• Impaired lymphatic drainage has been documented by lymphoscintigraphy in patients with facial edema (Nittner, 2010).

• None known

• Allergic angioedema
• Dermatomyositis
• Acne Rosacea
• Sarcoidosis
• Mycobacterial infection
• Lymphoma

• Natural history
• Relapses and recurrences are common.
• Difficult to define response to medical treatment.
• Medical therapy
• Intralesional steroid for focal inflammation.
• Oral corticosteroid for diffuse inflammation.
• Non-steroidal may spare systemic steroids.
• Metronidazole, dapsone, acyclovir, methotrexate and thalidomide have been tried with no consistent response (Elias, 2013).
• Radiation therapy
• None known
• Surgical options
• Surgical decompression of the facial nerve bony canal has been offered with limited success (Dai, 2014).
• Facial liposuction and muscle resections can reduce disfiguring facial edema (Tan, 2006).
• Eyelid debulking can improve cosmetic appearance (Cockerham, 2000).

• Melkersson described a female with facial edema and facial paralysis (1928).
• Rosenthal added another feature, lingua plicata (fissured tongue) (1931).
• Triad became known as Melkersson-Rosenthal syndrome (MRS).

• Cockerham KP, Hidayat AA, Cockerham GC: Melkersson-Rosenthal syndrome: New clinicopathologic findings in 4 cases. Arch Ophthalmol 2000; 118:227.
• Dai C, Li J, Yang S, et al: Subtotal facial nerve decompression for recurrent facial palsy in Melkersson-Rosenthal syndrome. Acta Otolaryngol 2014; 134:425.
• Demaria A, Zolezzi A, Passalacqua G, et al: Melkersson-Rosenthal syndrome associated with parvovirus b19 viraemia and haemphagocytic lymphohistiocytosis. Clin Exp Dermatol 2009; 34:e623.
• Elias MK, Mateen FJ, Weiler CR: The Melkersson-Rosenthal syndrome: A retrospective study of biopsied cases. J Neurol 2013; 260:138.
• Heinz C, Weinmann I: Bilateral conjunctival lesions in Melkersson-Rosenthal syndrome. Br J Ophthalmol 2001; 85:1260.
• Lin T, Chiang C, Cheng P: Melkersson-Rosenthal syndrome. J Form Med Assoc 2016; 115:583.
• Liu R, Yu S: Melkersson-Rosenthal syndrome: a review of seven cases. J Clin Neurosci 2013; 20:993.
• Melkersson E: Ett fall av recidiverande facialispares i samband med ett angioneurotiskt ödem. Hygiea 1928; 90:737.
• Nittner-Marszalska M, Krasnowska M, Solarewicz-Madejek K, et al: Melkersson-Rosenthal syndrome: lymphoscinigraphy-documented impairment and restoration of facial lymphatic drainage in the course of disease. Lymphology 2010; 43:34.
• Reddy DN, Martin JS, Potter HD: Melkersson-Rosenthal syndrome presenting as isolated eyelid edema. Ophthalmology 2017; 124:256.
• Rivera-Serrano CM, Man L, Klein S, Schaitkan BM: Merlkersson-Rosenthal syndrome: A facial nerve center perspective. J Plast Reconstr Aesth Surg 2014; 67:1050.
• Rosenthal C: Klinisch-erbbiologischer beitrag zur konstitutionspathologie. Gemeinsames auftreten von (rezidivierender familiarer) facialislahmung, angio- neurotischem gesichtsodem und lingua plicata in arthritismus-familien. Z Neurol Psychiatry 1931; 131:475.
• Tan O, Atik B, Calka O: Plastic surgical solutions for Melkersson-Rosenthal syndrome: facial liposuction and cheiloplasty procedures. Ann Pl Surg 2006; 56:268.
• Yeatts RP, White WL: Granulomatous blepharitis as a sign of Melkersson-Rosenthal syndrome. Ophthalmology 1997; 104:1185.