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 The glabella, in humans, is the area of skin between the eyebrows and above the nose. The term also refers to the underlying bone that is slightly depressed, and joins the two brow ridges. It is a cephalometric landmark that is just superior to ...

, upper

eyelid An eyelid is a thin fold of skin that covers and protects an eye. The levator palpebrae superioris muscle retracts the eyelid, exposing the cornea to the outside, giving vision. This can be either voluntarily or involuntarily. The human eye ...

s, nose, and/or cheeks.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 689. McGraw-Hill. .

Signs and symptoms

Persistent edema of rosacea is an uncommon

cutaneous condition A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of thi ...

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 Rosacea is a long-term skin condition that typically affects the face. It results in redness, pimples, swelling, and small and superficial dilated blood vessels. Often, the nose, cheeks, forehead, and chin are most involved. A red, enlarg ...

. 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.

Diagnosis

There are no specific laboratory findings associated with this condition, and the

histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vi ...

tends to be similar to that seen in rosacea. The differential diagnosis includes ''

acne Acne, also known as ''acne vulgaris'', is a long-term skin condition that occurs when dead skin cells and oil from the skin clog hair follicles. Typical features of the condition include blackheads or whiteheads, pimples, oily skin, and ...

vulgaris'', streptococcal cellulitis, and

Melkersson–Rosenthal syndrome Melkersson–Rosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip: cheilitis granulomatosis) and the development of folds and furrows in the tongue ( ...

, as these conditions can also rarely manifest with a similar edema, as well as other forms of rosacea,' ''

lupus erythematosus Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blo ...

'', and

sarcoidosis Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly a ...

Treatment

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 Isotretinoin, also known as 13-''cis''-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers ( squamous-cell carcinoma), and in t ...

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 Tetracyclines are a group of broad-spectrum antibiotic compounds that have a common basic structure and are either isolated directly from several species of ''Streptomyces'' bacteria or produced semi-synthetically from those isolated compounds. T ...

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.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 November 2010 ,

References

{{reflist Acneiform eruptions

Signs and symptoms

Diagnosis

Treatment

See also

References