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Editor,—The Melkersson syndrome is a rare granulomatous disease of unknown origin. The typical clinical picture consists of recurrent facial oedema associated with peripheral facial palsy and was first described by Melkersson in 1928.1 Three years later a fissured tongue called lingua plicata was added to the classic features by Rosenthal.2This clinical triad in patients with granulomatous cheilitis, facial palsy, and fissured tongue was first called Melkersson-Rosenthal syndrome (MRS) by Lüscher in 1949.3
We report on a patient with the typical clinical signs who had chronic bilateral conjunctival lesions. To our knowledge an association of conjunctival lesions with MRS has not been described previously.
A 64 year old man presented with a fissured tongue, recurrent painless facial oedema, especially of the eyelids, and facial flush for 6 years. A review of the other systemic diseases was unremarkable. He required blepharoplasty for the correction of lid malformation. The histopathological findings of the skin biopsy confirmed the clinical suspicion of MRS by the typical granulomatous infiltration (Fig 1A).
Furthermore, he complained bilateral conjunctival swelling had been present for 6 months. Visual acuity was 20/30. Slit lamp examination revealed a bilateral fleshy mass extending from the upper fornices to the limbus and conjunctival hyperaemia (Fig 1B). Motility of the eyeball was normal and an exophthalmus has not been present. In the magnetic resonance image (MRI) of the orbit a bilateral enlargement of lacrimal glands and a swelling of the lateral rectus and superior rectus muscle of the right eye were observed. Staging examinations for lymphoma or other malignancies were uneventful. Since differential diagnosis included a bilateral orbital lymphoma a conjunctival biopsy was performed. The histopathological examination of the conjunctival specimen by light microscopy revealed a subepithelial process. An infiltrate of small lymphocytes without any differentiation and with septate orientation was found (Fig 1C). By immunohistochemical examination no monoclonal pattern was determined, therefore, excluding a lymphoma.
Consequently, the patient was treated with clofazimine 100 mg daily. No progression in clinical findings was seen afterwards while he was on a maintenance dosage of the drug.
Disymptomatic or monosymptomatic clinical courses are frequently observed in MRS. As the symptoms rarely appear simultaneously MRS often can be diagnosed only by longitudinal follow up series.4Males and females are equally affected.5 Symptoms usually manifest during adolescence and have rarely been seen in childhood or individuals older than 50 years.6 The pathogenesis of MRS still remains obscure. Several predisposing factors have been considered such as heredity, infection, allergy, or derangement of cranial autonomic vasomotor innervation.1 7 Cranial nerve dysfunction (that is, trigeminal nerve), parasympathetic (flush, pain), and ocular involvement have been associated with MRS. Summarised ocular involvement includes granulomatous blepharitis, exophthalmus with lagophthalmus, and burning sensations, which may be related to exposure keratitis. In rare cases, palsies of the medial rectus muscle, papilloedema, and retrobulbar neuritis have been described.4-6 8 9
Conjunctival involvement has not been reported as yet. By conjunctival biopsy taken from our patient we have shown that conjunctival lesions may be present in MRS.
A satisfactory conservative therapy has not been established so far. The results of various modes of symptomatic treatment, including systemic or topical glucocorticosteroids, are questionable.10 They may reduce the patients' complaints, at least temporarily. Another treatment consists of clofazimine, which is an oral phenanzine. This drug had been useful in other conditions with granulomatous inflammation.9 Finally, a surgical excision of the masses had been suggested for granulomatous cheilitis or blepharitis, in order to improve the motility of the eyeball when exophthalmus occurred. Conjunctival biopsy with histopathological and immunohistochemical examination may be helpful to differentiate it from other lymphoid lesions.