ABSTRACT

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder characterized by recurrent facial palsy, orofacial swelling, and fissured tongue (lingua plicata). This case report presented a 16-year-old male who experienced his fourth episode of sudden-onset unilateral facial paralysis. Neurological examination revealed lower motor neuron-type facial weakness and a markedly enlarged tongue with deep fissures. Laboratory tests and imaging studies, including brain magnetic resonance imaging and systemic evaluations, were all within normal limits. Based on the presence of the classic clinical triad and the exclusion of other potential causes, a diagnosis of MRS was made. The patient responded well to corticosteroid therapy.

Introduction

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder, typically characterized by recurrent facial palsy, facial or lip swelling, and lingua plicata. Fewer than a third of patients present with all three symptoms and in most cases, symptoms occur progressively or partially (1-3).

The condition often begins in childhood or adolescence and follows a relapsing-remitting course. Swelling may resolve within hours or days initially, but may later become more persistent and even permanent due to granulomatous lymphatic obstruction. Meanwhile, facial palsy episodes are often temporary but may recur. Lingua plicata is usually asymptomatic and detected on physical examination (3, 4).

Although the etiology is unclear, proposed causes include genetic predisposition, familial clustering and possible chromosomal associations, immune dysregulation, and associations with Crohn’s disease, sarcoidosis, or orofacial granulomatosis triggered by hormonal changes have been reported in studies (5).

Diagnosis is made clinically, supported by ruling out infectious, neoplastic, or demyelinating causes. Imaging findings are typically normal, though in rare cases granulomatous inflammation may be detected (6).

Treatment is symptomatic. Systemic corticosteroids are generally the first line of therapy, while immunosuppressants or biologics like methotrexate or anti-tumor necrosis factor agents are considered in chronic or recurrent cases (3, 6). Surgical intervention is rarely required but may be considered for cosmetic deformity or chronic edema (2, 6).

Case Report

A 16-year-old male presented with left-sided facial palsy, which had developed suddenly over the course of one day. This was his fourth episode of unilateral facial weakness within the past three years. Each episode had previously responded well to a short course of corticosteroids, with near-complete recovery. He reported no other systemic symptoms, no recent infections, trauma, or vaccination.

Neurological examination revealed lower motor neuron-type facial weakness, impaired eye closure, and asymmetry of the mouth. Additionally, his tongue appeared firm, enlarged, and showed characteristic deep fissures (lingua plicata) (Figure 1).

Laboratory studies including vasculitis panel, acute phase reactants, and viral serologies (herpes simplex virus, Epstein-Barr virus, cytomegalovirus) were all within normal limits. Magnetic resonance imaging (MRI) of the brain and brainstem, as well as MRA, revealed no structural or inflammatory abnormalities. A chest computed tomography and gastrointestinal consultation were conducted to evaluate for systemic granulomatous diseases; both were normal.

The clinical triad of recurrent facial nerve palsy, orofacial swelling, and fissured tongue led to the diagnosis of MRS, a rare granulomatous disorder characterized by the classic triad of orofacial swelling, recurrent facial palsy, and fissured tongue (2, 6).

Informed consent was obtained from the participant for the study.

Discussion

MRS should be suspected in adolescents with recurrent facial palsy, especially when accompanied by lip/tongue swelling or fissured tongue. A normal MRI and laboratory work-up help exclude mimics such as multiple sclerosis, vasculitis, or infectious cranial neuritis.

Our patient displayed all Bell’s palsy diagnostic criteria along with fissured tongue. The wide range of conditions which may present as MRS includes thyroid orbitopathy and allergic reactions and angioedema and bacterial and viral and filarial infections and systemic lupus erythematosus and dermatomyositis and Bell’s palsy itself and Ramsay Hunt syndrome (7). This situation didn’t require a biopsy to confirm MRS diagnosis because all classic signs were present. The oral manifestations of Crohn’s disease and other inflammatory bowel diseases include lip swelling with fissures as well as mucositis and gingivitis and glossitis and the characteristic cobblestone appearance of oral mucosa (8). The available scientific evidence indicates that abnormal immune responses along with immune system imbalances and allergic tendencies affect people who develop MRS. The current medical literature does not identify any definitive treatment for this condition despite its management using short-term immunosuppressive medication (8). Medical practitioners use corticosteroids as initial treatment for MRS by administering them through injection into the lesion or systemically while decreasing medication doses over 3-6 weeks based on patient symptoms. Research shows that corticosteroid treatment leads to symptom improvement in 50-80% of patients while reducing the chance of reoccurrence to 60-75% (9). The treatment plan for this patient included methylprednisolone medication together with eye care for the affected side and physical therapy for facial palsy rehabilitation. The symptomatic treatment of eye care benefits from additional vitamins such as thiamine and niacin and riboflavin and pyridoxine and ascorbic acid and vitamin E. The treatment requires daily lubricating drop applications combined with overnight protective eye pad use because the eye can’t be closed completely. The recovery of facial palsy benefits from physical therapies that include exercise together with biofeedback and electrotherapy and massage and thermotherapy.