Facial edema revealing Melkersson-Rosenthal syndrome: a case report

J. OUJABER (1), B. NISRINE, Z. CHAHBI, S. HADDOURI, S. QACIF, M. ZYANI (2), K.
TOURABI (3), R. SIRBOU* (1)
Page No. 53-59

Abstract

Melkersson-Rosenthal syndrome (SMR) is a rare entity. It is a granulomatosis Facial affecting both sexes

with a triad of bucco-facial manifestations: mucocutaneous edema of the face, recurrent peripheral facial

paralysis and a plicature tongue. . Hubschman in 1894 described the first case without being able to

establish the relationship between the three symptoms. In 1928, Melkersson made the link between

macrochemistry and facial paralysis, then Rosenthal identified the three signs that constitute the

characteristic triad ). Through this observation, we present the clinical and therapeutic features of this

pathology 46 year old patient consults in the emergency for the sudden appearance of edema of the face, a

feeling of numbness and a swelling affecting the labial region, attributed to a food allergy according to the

patient. An edema covering the middle floor of the face and the right and left latero-mandibular region The

charles Bell negative sign, negative eyelash sign, no hypoesthesia The endo buccal examination reveals a

pleated appearance of the tongue, gingival inflammation, the absence of dental infectious . Objective joint

osteo examination of diffuse myalgia, poly arthralgia of large inflammatory joints. A salivary gland biopsy

confirmed the diagnosis, finding a histological appearance compatible with chronic granulomatous

epitheloid and giganto-cellular sialdenitis in the absence of caseous necrosis, on an inflammatory

lymphocytic background.. A protocol adopting a monthly intra-lesional injection of 40 mg of triamcinolone

acetonide (Kénacort Retard®) in each lip was undertaken giving satisfactory results.