- International Classification of Diseases (ICD)
- ICD 11: BD93.1Y: Lymphoedema secondary to other specified cause
Morbihan disease (MD), also known as solid persistent facial edema, lymphedema rosacea, morbus Morbihan and Morbihan syndrome, is a rare condition characterized by chronic, progressive, non-pitting edema (+/- erythema) of the upper two-thirds of the face, notably the periorbital tissue, forehead, glabella, nose, and cheeks, that may result in facial disfigurement and visual field narrowing   
History of the disease
MD affects the upper two thirds of the face, including:
- Periorbital region: both preseptal and pretarsal tissue
The cause of MD remains unknown. Many authors propose that MD is caused by lymphatic dysregulation, chronic inflammation, or both. The hypotheses fall under several categories:
- There is an imbalance between lymphatic production and drainage 
- Mast cells obstruct dermal lymphatics or cause dermal fibrosis. 
- Peri and intra-lymphatic granulomas obstruct lymphatic drainage 
- Chronic inflammatory mediators, released due to underlying autoimmune dysregulation or infection, cause vascular wall damage and breakdown of connective tissue within the dermis leading to persistent exudation and resultant edema.
- Contact urticaria, in response to topical irritants, triggers local inflammation resulting in insufficient lymphatic drainage in individuals with pre-existing lymphatic drainage defects
The relationship between Morbihan disease, rosacea and acne has been theorized due to histopathologic similarities.  The limitation of this hypothesis, however, is that many patients with MD do not have rosacea or acne. The association between rosacea, acne and MD remains unclear.
The most common histopathologic findings reported include:
- Perivascular and perifollicular lymphocytic and histiocytic infiltration
- Presence of mast cells
- Perifollicular fibrosis
- Dilated lymphatic channels in the dermis
- Dermal edema
Other, less commonly described, findings include:
- Perifollicular and peri-lymphatic epithelioid granulomas
- Lymphatic histiocytic infiltration
- Dilated blood vessels
- Plasma cells
- Giant cells
- Increased collagen spacing and thickness
- Sebaceous gland hypertrophy/hyperplasia
- Chronic folliculitis
The incidence and prevalence of MD are unknown.
- Male > Female
- Middle age
- Ethnicity: Caucasian/white
- Most of the cases described in the literature were of Caucasian individuals, followed by Asian individuals (Japanese, Chinese, Korean). Few reports in other ethnicities.
- Other: exposure to sun and woodworking dust.
Patients note an insidious onset of upper facial swelling. Pertinent points on history include:
- Swelling of upper face, with or without redness
- Insidious onset, progressive 
- Non-painful, non-pruritic 
- Possible visual impairment from increased lacrimation or mass effect causing ptosis and visual field narrowing 
- Edema that is generally not position dependent, although may be described as worse in the morning 
- May be associated with hot sensation of face, facial flushing , or psychosocial distress due to cosmetic disfigurement
Physical examination findings include:
- Non-pitting, solid edema affecting the upper two-thirds of the face.
- Erythema of the overlying skin. Erythema is typically ill-defined, present in discrete patches, or solitary plaques.
- Findings may be symmetric or asymmetric, unilateral or bilateral 
- Typically, preserved visual acuity and eye exam within normal limits 
- May lead to visual impairment / visual field narrowing due to ptosis from mass effect and lacrimation 
- Can cause significant facial disfigurement 
- Peau d’orange skin texture (few cases) 
- Bilateral chemosis of the anterior segment has been reported in one case 
- Signs of rosacea, telangiectasia, papules, pustules, granulomas, nodules 
There are no diagnostic criteria for MD. It is a diagnosis of exclusion. Investigations are used to rule out other causes of facial edema and are ordered at the discretion of the healthcare provider based on the patient’s specific presentation (see differential diagnoses below).
The mainstays in investigations are bloodwork, radiographic imaging, and biopsy. Laboratory bloodwork can rule out systemic disease. Preoperative orbital computed tomography (CT) can be used to assess for orbital tumours. Biopsy of the skin can be used to rule out other dermatologic disease.
|Investigations to consider||Rationale & Findings|
|Blood work||Note: Laboratory investigations are generally normal in MD
|Differential diagnosis of chronic facial edema / eyelid swelling |
There are no systemic manifestations of MD.
Management of eyelid edema
There is no gold standard for the treatment of this rare disease.
Patients can be recommended avoidance potential triggers (see “prevention”).
Interventions result in variable clinical improvement. Recurrence or progression can be seen after treatment discontinuation. A combination of interventions have been used with some success, although a systematic review found no superior effect with combination therapy on outcomes and a greater risk of adverse effects. Some case reports and case series have shown promising results.
|Management in the literature
It has been hypothesized that medical therapy often fails due to impaired local delivery systems at the site of chronic inflammation and interstitial edema in MD patients. Erythema and inflammatory signs may respond to medications, but edema often persists. Combining surgical debridement with anti-inflammatory medical therapy may improve treatment response.
Future considerations for management include the use of immunosuppressant medications to target lymphocyte populations. Azathioprine and omazilumab have been suggested as potential therapies.
Without treatment, MD is unlikely to resolve spontaneously. The condition is localized to the face and has no known systemic manifestations. MD is often refractory to treatment; however, most cases show at least partial response to conventional treatment. A review article on the topic suggests patients may benefit from 4- to 6- months of tetracycline-based antibiotics with the risk of side effects weighed against the benefits of treatment. Oral steroids were correlated with recurrence or progression. Male gender correlated with lack of complete response to treatment. Patients who undergo debulking respond to treatment although response may be partial. Approximately 10% of patients have recurrence or progression of disease.
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