Persistent unilateral orbital and eyelid oedema as a manifestation of Melkersson-Rosenthal syndrome

B. Cocuroccia, E. Gubinelli, G. Annessi, G. Zambruno, G. Girolomoni

Research output: Contribution to journalArticlepeer-review

Abstract

Melkersson-Rosenthal syndrome (MRS) is a complex neuromucocutaneous disorder characterized by localized orofacial oedema and cranial nerve dysfunction, frequently associated with minor signs, including furrowed tongue. Complete forms are rare whereas mono- and oligosymptomatic variants are more common. A 71-year-old man presented with a 2-year history of relapsing and progressively persistent oedema of the right eyelids and periorbital region. A fissured tongue and telangiectatic rosacea had been present since the age of 50 and 60 years, respectively. The patient was also affected by essential hypertension and diabetes mellitus. A skin biopsy showed a marked upper dermal oedema, and small epithelioid cell granulomas arranged in perivascular and perilymphatic location. Collections of small epithelioid cells were occasionally observed within lymphatic spaces. No acid-fast bacteria, fungi or foreign bodies were detected. Intralesional corticosteroids induced transient improvement, whereas minocycline, clofazimine and dapsone have been ineffective. MRS may present with unilateral eyelid and periorbital swelling. Differential diagnoses of such cases may include a variety of cutaneous, ophthalmic and systemic diseases.

Original languageEnglish
Pages (from-to)107-111
Number of pages5
JournalJournal of the European Academy of Dermatology and Venereology
Volume19
Issue number1
DOIs
Publication statusPublished - Jan 2005

Keywords

  • Eyelid oedema
  • Granulomatous blepharitis
  • Melkersson-Rosenthal Syndrome
  • Orofacial granulomatosis

ASJC Scopus subject areas

  • Dermatology
  • Infectious Diseases

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