Ocular Pathology

Anatomy and pathology of the human eye. Use it to review eye pathology for Ophthalmology Board Review or OKAP.

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Wednesday, September 13, 2006

What is Melkersson-Rosenthal syndrome?

Melkersson Rosenthal Syndrome
First described in 1928, the syndrome is characterized by orofacial edema swelling, facial nerve paralysis and a fissured tongue. Histologically there are dilated lymphatics with perivascular and occasional intralymphatic vascular granulomatous inflammation.
Incidence/Prevalence: More common in females than males by a ratio of 3:1. This syndrome is quite rare and seems to be more prevalent in Europe than in the U.S.
Etiology: The etiology of Melkersson-Rosenthal syndrome remains controversial. No convincing evidence exists for any infectious, allergic or hereditary origin. There is a familial tendency and some authors claim there is an autosomal dominant pattern of inheritance with variable penetrance.

Clinical Findings: Generally occurs more commonly in women. The syndrome does occur in children but is much more common in adults. Non-pitting facial edema, facial nerve paralysis (left side of face in the image to the left and a fissured tongue ( arrows 1 below) form the classic triad. The upper lid is more often involved than the lower lid (see image to the left). The facial nerve paralysis (shown in the image) is often delayed in onset, years after the facial edema and is indistinguishable from Bell’s palsy. The onset of the disease episode is sudden and may last hours to weeks.

In addition migraine headaches and constitutional symptoms such as hyperpyrexia have been described. Fibrosis may ensue. Distinguished from angioedema, Melkersson-Rosenthal syndrome lasts longer and is unresponsive to antihistamines.
Histopathology: A skin biopsy is usually performed to aid in the diagnosis. Typical histopathological findings include edema, non-caseating epithelioid cell granulomas, multinucleate Langerhans-type giant cells, perivascular mononuclear infiltration and fibrosis. The low power image of the skin shows some thickening of collagen bundles (fibrosis) and an increased distance between them (suggestive of edema). There is a dilated lymphatic in the deep dermis that is filled with epithelioid histiocytes. (Click to enlarge the photograph.) There are sparse extravascular infiltrates of lymphocytes and histiocytes in this image. Lymphocytic and histiocytic infiltration of lymphatics may be seen in the early stages of the disease. At higher magnification one sees that the intralymphatic infiltrate is composed of single epithelioid histiocytes (arrow 3) with bean shaped nuclei that are eccentric in the cells and abundant foamy cytoplasm. Note that the infiltrate is accompanied by some lymphocytes.
One can see the spindle cells lining the blood vessels of these lymphatics (arrow 2). Intralymphatic and perilymphatic granulomatous infiltrates (arrow 4) are very characteristic of this disorder.
Treatment: Some authors have had success with short doses of corticosteroids during attacks but otherwise the treatment is largely symptomatic.
Prognosis: Recurrent episodes are likely.

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