Persistent edema of rosacea (also known as chronic upper facial erythematous edema, Morbihan's disease or rosaceous lymphedema) is a hard, nonpitting
edema
Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue (biology), tissue. Most commonly, the legs or arms are affected. S ...
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 ...
, 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. "Palpebral ...
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 this sys ...
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, en ...
. 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 that studies the microscopic anatomy of biological tissue (biology), tissues. Histology is the microscopic counterpart to gross anatomy, which looks at large ...
tends to be similar to that seen in rosacea. The
differential diagnosis
In healthcare, a differential diagnosis (DDx) is a method of analysis that distinguishes a particular disease or condition from others that present with similar clinical features. Differential diagnostic procedures are used by clinicians to di ...
includes ''
acne
Acne ( ), also known as ''acne vulgaris'', is a long-term Cutaneous condition, skin condition that occurs when Keratinocyte, dead skin cells and Sebum, oil from the skin clog hair follicles. Typical features of the condition include comedo, ...
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
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, blood cells, heart, ...
'', and
sarcoidosis
Sarcoidosis (; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of White blood cell, inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph n ...
.
Treatment
Most cases of this condition tend to be recalcitrant to treatment, with topical and oral
antibiotic
An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy ...
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 used to treat skin diseases like harlequin-type ichthyosis, and lamellar ichthyosis, and severe cystic acne or moderat ...
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. ...
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 , ]
See also
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the Human body, body and composed of Human skin, skin, hair, Nail (anatomy), nails, and related muscle and glands. The major function o ...
References
{{reflist
Acneiform eruptions