Morbihan Disease: A Rare Variant of Rosacea

Shim, Woo Haing; Jwa, Seung Wook; Song, Margaret; Kim, Hoon Soo; Ko, Hyun Chang; Kim, Byung Soo; Kim, Moon Bum

Abstract

Morbihan disease, also known as 'rosacea lymphedema' or 'solid persistent facial edema' was first reported by Degos, in 1957, for describing persistent lymphedema on the upper half of the face in association with rosacea. Although the pathogenesis of Morbihan disease is not established well, two hypotheses were proposed; firstly, the hypothesis of lymphatic obstruction by destruction of vessel supporting connective tissue (e.g. elastin), and secondly, the hypothesis of mast cell-induced fibrosis. Herein, we report a 71-year-old man who had 9-months history of persistent bilateral edema of the eyelids. The histopathologic exam from edematous eyelid revealed marked dermal edema, dilated vascular structure with perivascular mixed cellular inflammatory infiltration. Moreover, focal granulomatous infiltration adjacent to a vascular structure and numerous mast cells were also identified. This case is the first reported case of Morbihan disease in Korean dermatological literature. Furthermore, the histopathologic findings of this case support both of the two hypotheses for development of Morbihan disease.