Melkersson–Rosenthal Syndrome in Childhood: Report of Three Paediatric Cases and a Review of the Literature

Abstract

Melkersson–Rosenthal syndrome (MRS) in children is a rare condition, clinically characterised by a triad of synchronous or metachronous symptoms: recurrent peripheral facial palsy, relapsing orofacial oedema, and a fissured tongue; the most recent review published on the topic has reported 30 published patients. The aetiology of this disease is still unclear. However, genetic factors, as well as alterations in immune functions, infections, and allergic reactions have been postulated. We report three children suffering from MRS and perform a literature review of paediatric cases. Taking into account that clinical and laboratoristical criteria for the diagnosis of MRS are lacking, this syndrome is probably underestimated, and we suggest increasing awareness of such a rare syndrome. Close multidisciplinary follow-up of these children with a team composed by paediatricians, neurologists, neuro-ophthalmologists, dermatologists, and otolaryngologists is crucial to guarantee exhaustive management and treatment success, while minimising relapses.

<p>Management Strategies of Melkersson-Rosenthal Syndrome: A Review</p>

“…Edema is painless, non-pitting and most commonly seen in the upper limbs, but may also be seen in lower lip, tongue, buccal area, eyelids, neck, gingiva, nose, upper arms, genitalia, and the larynx. 4,5,7 Partial or non-resolution of edema may lead to fibrosis and may lead to permanent disfiguration of the face.…”

“…The etiopathogenesis of this rare pediatric disorder has been linked to HSV1 infections, hereditary granulomatous disorders, mycobacterial infections (tuberculosis, leprosy), chronic infections, Down's syndrome, psoriasis, thyroiditis, multiple sclerosis, keratitis, Wegener's granulomatous, diabetes mellitus, sarcoidosis, ulcerative colitis, and allergic disorders. 5,7,14 Influenza respiratory infection may trigger a relapse of MRS. Gene mutations causing UNC-93B deficiency may predispose to HSV-1 infections. 15 This deficiency selectively impairs immunity against HSV-1 but not against other organisms.…”

“…Traditionally, corticosteroids have been the mainstay of treatment. 7 There are no randomized trials to suggest the corticosteroid type and duration that should be used. Therapy with corticosteroids leads to improvement in 50-80% of patients and reduced relapse frequency by 60-75%.…”

“…Therapy with corticosteroids leads to improvement in 50-80% of patients and reduced relapse frequency by 60-75%. 7 Typically, oral corticosteroids are used for 1 week and tapered over 2 weeks. High-dose pulse methylprednisolone has been used in severe cases.…”

“…4,5 MRS is rare in children, and only a handful of 30 cases have been described. 6,7 Onset in children is common between 7 and 12 years of age, with the youngest child described at 22 months of age. The diagnosis is frequently delayed with the median time to the diagnosis being 4-9 years.…”