Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis

Abstract

Morbihan disease is a rare complication of rosacea, characterized by persistent lymphoedema on the upper half of the face, occurring during the chronic clinical course of rosacea. This refractory condition has been also designated as 'rosacea lymphoedema' and 'solid persistent facial oedema of rosacea'. We report a patient with Morbihan disease showing persistent lymphoedema on the upper half of the face accompanied by unique histological findings of striking dermal dilated lymphatics and damage of the lymphatics at the site of the adjacent epithelioid cell granulomas, with histiocytes bulging into the lymphatic lumen. The marked epithelioid cell granulomas forming around dermal lymphatic vessels with subsequent lymphatic damage and luminal obstruction by histiocytic infiltration may account for the development of lymphoedema in this patient.

Successful Treatment of Morbihan Disease with Long-term Minocycline and its Association with Mast Cell Infiltration

“…Marked infiltration of mast cells was observed in our case and in some previously reported cases (7,13), but not in other cases (1,2). The difference may depend on the stage of the disease and the way skin biopsies were taken.…”

“…Morbihan disease is characterised by persistent lymph oedema on the upper half of the face, which is also referred to as rosaceous lymphoedema and solid persis tent facial oedema (1). The term Morbihan disease was derived from the French region where the first patient was observed (2,3).…”

Successful Long-term Use of Oral Isotretinoin for the Management of Morbihan Disease

“…The word Morbihan references the French district where the condition was first reported; however, MD has gone by several other names, including rosaceous lymphedema, solid persistent facial edema, and morbus Morbihan. [1][2][3] We report 5 cases of MD that were successfully treated with long-term oral isotretinoin therapy along with a literature review of current options for the treatment of MD.…”

Rosazea 2009

“…Eine mögliche Erklärung der Entstehung der Ödeme beim M. Morbihan könnten 7 epitheliale Zellgranulome sein, die sich um die dermalen Lymphgefäße bilden und dadurch den Lymphabfluss behindern, sowie eine zusätzliche luminale Obstruktion durch Histiozyten [26].…”