Cheilitis granulomatosa associated with melkersson-rosenthal syndrome

Abstract

Melkersson-Rosenthal syndrome (MRS) consists of persistent or recurrent orofacial edema, relapsing facial palsy and fissured tongue. The complete triad of symptoms is uncommon, varying from 8 to 25%. The presentation of only one symptom is more common. The most frequent complaint is facial edema and enlargement of the lips. We describe a case of a 17-year-old Brazilian girl with limited edema of the lower lip and fissured tongue due to MRS. Her complaints had started two years before. She referred previous clinical treatments without success. We proposed intralesional injection of triamcinolone at 20 mg every 15 days associated with oral clofazimine at 50 mg/day for three months. The lip became normal after four triamcinolone injections. Recent studies have considered MRS a granulomatous disease, and possibly the initial presentation of Crohn's disease in orofacial area of some patients. MRS patients, therefore, should be screened and monitored for gastrointestinal symptoms. Corticosteroid treatment seems to be effective in reducing lip enlargement. We discuss the clinical features of this disease, the treatment, and the importance of corticosteroid therapy in cases of MRS-related facial palsy.

Successful treatment of cheilitis granulomatosa with intralesional injection of triamcinolone

“…Regarding familiar occurrence of deafness in association with skin diseases, Heathcote et al reported a three generation family with an autosomal dominant syndrome of sensorineural hearing loss and palmoplantar hyperkeratosis, which has been regarded as a distinct clinical entity (MIM number 148350). 5 Although previously not reported in association with sensoneurinal deafness, it is possible that EC and TS resulted from the same underlying genetic abnormalities that lead to hyperproliferative tendency of this patient's epidermis leading to plugging of the follicular orifices and hearing impairment. Speculation is based on very similar skin phenotype in close relatives connected with hearing impairment.…”

“…Sussman et al 4 showed complete remission in five of the 11 patients with CG following the treatment. Ratzinger et al 5 observed complete or partial response in majority of patients treated with clofazimine. Unfortunately we have no experience with this drug.…”

“…1 In the literature, we found a case described of unsuccessful treatment, 2 and a case of successful treatment of MRS-related headache with dapsone, 3 while the use of intralesional triamcinolone alone is widely described. [4][5][6][7] A 50-year-old male patient presented to us with a permanent swelling of the upper lip, with a slight erythematic aspect, more evident at the right side; on palpation, there was an increased consistency, which felt granulose, and no pain was evoked, and the swelling had begun about 2 years ago (Fig 1).…”

Oral Diseases Associated with Gastrointestinal Disorders

“…Also, localization on the upper lip causes a proboscis-like malformation called 'tapirus mouth' [46]. Gonçalves et al [47] reported a 17 years old patient with continuing swelling of the lips with fissured tongue due to MRS. Recent studies have considered MRS as the first sign of Crohn's disease, therefore, these patients should be screened and observed for gastrointestinal symptoms.…”

Melkersson-Rosenthal syndrome

“…[ 1 ] However, the monosymptomatic form is more common and typical manifestation is facial edema and/or enlargement of lips. [ 2 ]…”