Complete Melkersson-Rosenthal Syndrome in a Patient with Crohn’s Disease

Melkersson-Rosenthal syndrome Crohn’s disease Orofacial oedema Prof. Michele Fimiani, Istituto di Clinica Dermatologica, Università degli Studi di Siena, Policlinico ‘Le Scotte’, I-53100 Siena (Italy), Tel. 577 40190, Fax 577 44238 Melkersson-Rosenthal syndrome (MRS) is an uncommon condition, well known in terms of clinical features and course but of uncertain aetiopathogenesis [1-3]. The histopathology of the lesions, characterized by non-caseating epithelioid granulomatous inflammation, suggests a possible relation with sarcoidosis and overall with Crohn’s disease (CD) [4]. A 23-year-old woman had CD at the age of 10 years, and for this reason she underwent right hemicolectomy in 1984. In the following years, clinical examination, laboratory tests and scintigraphy with labelled granulocytes did not detect any signs of intestinal relapse. Seven years after the operation, the patient was in good health but developed recurrent oedema of the upper lip. These episodes progressively increased in frequency and intensity, eventually involving most of the right side of the face. When examined by us, the patient had persistent swelling of the right side of the face, partially involving the upper lip. The tongue and oral mucosa were spared. She complained of dysaesthesia of the homolateral side of the face and devastating psychological effects due to her appearance. Histological examination of a biopsy specimen of the oral mucosa showed many non-necrotizing granulomas consisting of epithelioid cells, giant cells and lymphocytes throughout the dermis. Electromyographic examination showed a slight reduction in amplitude of evoked potentials of the right facial nerve. Routine laboratory tests were normal. Chest X-ray and assay of serum calcium, lysozyme, ß2-microglobulin and angiotensin-converting enzyme were normal. No clinical signs of intestinal disease were present. Gastro-intestinal radiological examination and sigmoidos-copy were both normal. A treatment consisting of 100 mg/day azathi-oprine and 20 mg/day 6-methylprednisolone during 1 year, the latter during episodes of oedema, had no substantial effect on the course of the disease. It is widely accepted that MRS can only be diagnosed with certainty when there is at least one of the so-called major signs: recurring orofacial oedema and/or facial paralysis associated with histological features of sarcoid-like granuloma [1]. When there is one or more minor signs (plicate tongue, histological aspecific oedema, involvement of cranial nerves, migraine, vegetative orofacial symptoms, psychiatric symptoms during the episodes of oedema), MRS can