Persistent edema of rosacea
Persistent edema of rosacea (also known as "Chronic upper facial erythematous edema," "Morbihan's disease," or "Rosaceous lymphedema") is a hard, nonpitting edema found on the areas involved, those mainly being the forehead, glabella, upper eyelids, nose, and/or cheeks.
Signs and symptoms
Persistent edema of rosacea is an uncommon cutaneous condition characterized by a hard, nonpitting edema restricted to the forehead, glabella, upper eyelids, nose, and cheeks. This condition is also known as chronic upper facial erythematous edema, Morbihan's disease, morbus Morbihan, and rosaceous lymphedema. Despite the name, it is unclear whether this condition is a distinct disease or a rare complication of rosacea. Generally, there are few symptomatic complaints aside from redness and facial contour changes. The edema typically worsens slowly over months to years and is often on a background of chronic inflammation.
There are no specific laboratory findings associated with this condition, and the histology tends to be similar to that seen in rosacea. The differential diagnosis includes acne vulgaris, streptococcal cellulitis, and Melkersson–Rosenthal syndrome, as these conditions can also rarely manifest with a similar edema, as well as other forms of rosacea, lupus erythematosus, and sarcoidosis.
Most cases of this condition tend to be recalcitrant to treatment, with topical and oral antibiotic regimens commonly used for rosacea generally being ineffective. For the severe forms of this disease, oral isotretinoin therapy has been used, and the condition responds well to doses of 0.5 to 1 mg/kg/day. However, unlike in the treatment of acne vulgaris, lasting responses to isotretinoin generally do not occur, and long-term maintenance therapy with oral tetracyclines is usually necessary. Eyelid reduction surgery has also been reported to help with the cosmetic appearance of the condition, but does not alter the disease progression.
- Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 689. McGraw-Hill. ISBN 0-07-138076-0.
- Ajith C, et al. Granulomatous rosacea mimicking eyelid dermatitis. > OUCH! Indian J Dermatol Venereol Leprol. 2005;71(5):366-5.
- Freedberg IM, et al. Fitzpatrick's Dermatology in General Medicine. Sixth Edition. McGraw-Hill, 2003:689-90.
- Lamparter J, et al. Morbus Morbihan : A rare cause of edematous swelling of the eyelids. Ophthalmologe. 2010;107(6):553-7.
- Nagasaka T, et al. Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis. Clin Exp Dermatol. 2008;33(6):764-7.
- Wohlrab J, et al. Persistent erythema and edema of the midthird and upper aspect of the face (morbus morbihan): evidence of hidden immunologic contact urticaria and impaired lymphatic drainage. J Am Acad Dermatol. 2005;52(4):595-602.
- Harneet Ranu, Joyce Lee and Tan Hiok Hee. "THERAPEUTIC HOTLINE: Successful treatment of Morbihan's disease with oral prednisolone and doxycycline" (pages 682–685), Dermatologic Therapy. Article first published online: 3 NOV 2010 | doi:10.1111/j.1529-8019.2010.01373.x