Morbihan Disease

From EyeWiki
Assigned editor:
Assigned status Up to Date
 by Natalie Homer, MD. on June 14, 2023.

Disease Entity

  • 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 [1] [2] [3]

History of the disease

MD was first observed in the 1950s. [4] [5] It was named after Morbihan, a department in Brittany, France where the findings were described by a dermatologist, Dr Robert Degos.


MD affects the upper two thirds of the face, including:

  • Forehead
  • Glabella
  • Periorbital region: both preseptal and pretarsal tissue
  • Cheeks
  • Nose


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:

  1.   There is an imbalance between lymphatic production and drainage [6][7]
  2.   Mast cells obstruct dermal lymphatics or cause dermal fibrosis. [8][9][3]
  3.   Peri and intra-lymphatic granulomas obstruct lymphatic drainage [10][11]
  4.   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.[12][13][14][15][16]
  5. Contact urticaria, in response to topical irritants, triggers local inflammation resulting in insufficient lymphatic drainage in individuals with pre-existing lymphatic drainage defects[7]

The relationship between Morbihan disease, rosacea and acne has been theorized due to histopathologic similarities. [14] The limitation of this hypothesis, however, is that many patients with MD do not have rosacea or acne.[1][2][17] The association between rosacea, acne and MD remains unclear.


The histopathology is non-specific in MD. [9][18][19][11][20][1][15][21][2][14][22][23][10][16][24][25][8][3]

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
Histopathology of upper eyelid tissue in a patient with Morbihan Disease using hematoxylin and eosin (H&E) stain. Findings are non-specific. (A) 10x. Dermal edema with dilated lymphatics. (B) 10x. Perifollicular fibrosis, dermal edema, and dilated vessels. (C) 20x. Dilated lymphatics and dermal edema. (D) 40x. Dermal edema and mast cells. Slides provided by David Plemel, MD.

Other, less commonly described, findings include:

  • Perifollicular and peri-lymphatic epithelioid granulomas
  • Lymphatic histiocytic infiltration
  • Telangiectasia
  • Dilated blood vessels
  • Plasma cells
  • Giant cells
  • Neutrophils
  • Increased collagen spacing and thickness
  • Sebaceous gland hypertrophy/hyperplasia
  • Chronic folliculitis


The incidence and prevalence of MD are unknown.

Risk factors

  • Male > Female
    • Despite previous literature indicating MD affects women more than men, which would be in keeping with rosacea, recent studies have found that there are more cases described in men than in women. [1][3]
  • Middle age
    • Most common ages 40-60, although cases reported between ages 14-88.[1][3]
  • 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.[1]
  • Other: exposure to sun and woodworking dust.[26][27]


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 [18]
  • Non-painful, non-pruritic [1]
  • Possible visual impairment from increased lacrimation or mass effect causing ptosis and visual field narrowing [19][27][2]
  • Edema that is generally not position dependent, although may be described as worse in the morning [19]
  • May be associated with hot sensation of face, facial flushing [28][3], or psychosocial distress due to cosmetic disfigurement[1]

Physical examination

Physical examination findings include:

  • Non-pitting, solid edema affecting the upper two-thirds of the face.
    • Locations most commonly involved include eyelids, forehead, glabella, and cheeks[10][18]
    • The edema has been described as hard, or woody, with a smooth surface[26]
    • Edema may initially be pitting, and become non-pitting over time[1]
  • Erythema of the overlying skin.[1] Erythema is typically ill-defined, present in discrete patches, or solitary plaques.
  • Findings may be symmetric or asymmetric, unilateral or bilateral [1][11]
  • Typically, preserved visual acuity and eye exam within normal limits [20][23]
  • May lead to visual impairment / visual field narrowing due to ptosis from mass effect and lacrimation [19][27][2]
  • Can cause significant facial disfigurement [1]
  • Peau d’orange skin texture (few cases) [27][19][20]
  • Bilateral chemosis of the anterior segment has been reported in one case [1]
  • Signs of rosacea, telangiectasia, papules, pustules, granulomas, nodules [3][1][29]


There are no diagnostic criteria for MD. It is a diagnosis of exclusion.[3] 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).

Laboratory test

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
  • Complete blood count (CBC) for blood cell diseases
  • Inflammatory markers, C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), for inflammatory diseases
  • IgG4 for IgG4 disease
  • Angiotensin converting enzyme (ACE) and calcium for sarcoid
  • Albumin, bilirubin, creatinine, estimated glomerular filtration rate (eGFR) and alkaline phosphate (ALP) to assess for liver or renal anomalies.
  • Antinuclear antibody (ANA) and antineutrophil cytoplastic antibodies (ANCA) to screen assess for autoimmune markers
  • Thyroid stimulating hormone (TSH) and T3/T4 to assess for thyroid dysfunction.
  • Thyroid antibodies to assess for autoimmune thyroid disease.
  • Orbital CT
    • To show preseptal, pretarsal and subcutaneous soft tissue swelling while ruling out orbital pathology.[20][1][23][30]
  • Chest x-ray or thoracic CT
    • To assess for hilar lymphadenopathy in sarcoidosis
  • Ultrasound and doppler flowmetry
    • Used in research studies to show insufficient lymphatic drainage[18]
  • Indocyanine green (ICG) lymphangiography
    • Used in research studies to identify locations of lymphatic vessels[7]

  • Histology [15][21][25][3]
    • Hematoxylin and eosin stain → microanatomy
    • Verhoeff-Von Gieson stain → elastic fibres
    • Masson’s trichrome stain → collagen
    • Giemsa stain, toludine blue → mast cells
  • Immunohistochemistry [11][15][2]
    • D2-40 / CD31 → lymphatic channels / specific for lymphatic endothelial cells
    • CD68 → histiocyte infiltration
    • CD117 / C-kit → mast cells
  • Others to consider
    • Evaluation for demodex mites [1][19]
    • Stains for fungi and mycobacteria (Ziehl-Neelson stain) [30][23][19]
    • Stains for mucin (colloidal iron, Periodic acid-Schiff) [25][19]

Differential diagnosis

Differential diagnosis of chronic facial edema / eyelid swelling [31][23][20][15][11][19]
  • Acne rosacea
  • Acne vulgaris
  • Systemic lupus erythematosus
  • Dermatomyositis
  • Angioedema
  • Allergic dermatitis
  • Chronic actinic dermatitis
  • Sarcoidosis
  • Amyloidosis
  • Erysipelas
  • Herpes zoster
  • Leprosy
  • Leishmaniasis
  • Lymphoma
  • Angiosarcoma
  • Other tumours
  • Trauma
  • Irradiation
  • Orofacial granulomatosis
    • Melkersson-Rosenthal Syndrome (including all or part of the triad: facial palsy, facial edema, fissured tongue)
  • Congenital
  • Hypothyroidism, myxedema
  • SVC syndrome
  • Medications that can induced similar clinical signs: barbiturates, chlorpromazine, diltiazem, isotretinoin


Systemic associations

Morbihan disease may be associated with rosacea, acne or both. [20][14][11][15]

Systemic manifestations

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.[1] A combination of interventions have been used with some success,[22][32][29][33][34] although a systematic review found no superior effect with combination therapy on outcomes and a greater risk of adverse effects.[1] Some case reports and case series have shown promising results.

Management in the literature
  • Most commonly used management strategies are highlighted in bold
  • Intralesional steroid injection (triamcinolone) [15][22][24][3]
    • The authors would suggest injecting steroids periorbitally, deep to the orbicularis oculi muscle.
  • Lymphedema management including compression therapy, manual lymphatic drainage, skin care [7][32][35]
  • Other (less effective or less studied): Topical metronidazole and sulfur wash [1]
  • Isotretinoin [26][29][32][16][33][6][32]
    • Median dose of 40mg PO daily for a duration of 6 months.[1]
    • Tetracyclines including doxycycline or minocycline [1][36]
    • Medial daily dose 200mg daily with duration correlating to treatment response; duration to partial response 3.0 month compared to 6.5 months for complete response [1]
  • Other (less effective or less studied):
    • Metronidazole, Corticosteroids [23][28]
    • Diuretics including furosemide and spironolactone [31]
    • Antihistamines including ketotifen [34]
    • Clofazimine or Thalidomide [16]
    • Omalizumab [37]
  • Special considerations:
    • A review in 2019 found that oral steroids do not impact outcomes and are correlated with recurrences or progression [1]
    • Isotretinoin and tetracyclines should not be combined as this has been reported to increase the risk of idiopathic intracranial hypertension [38]
    • Authors have reported good outcomes using a combination of ultra-low-dose isotretinoin with antihistamines [33]
Alternative therapy
  • Tripterygium wilfordii [39]
Preoperative photographs of a patient with Morbihan syndrome (above). There is decreased periocular soft tissue swelling after upper eyelid blepharoplasty with injection of steroids into all four eyelids (below). Photographs provided by Karim G Punja, MD. Patient consent was obtained for the publication of these photos on EyeWiki.

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.[15] Erythema and inflammatory signs may respond to medications, but edema often persists.[15] Combining surgical debridement with anti-inflammatory medical therapy may improve treatment response.[15]

Future considerations for management include the use of immunosuppressant medications to target lymphocyte populations. Azathioprine and omazilumab have been suggested as potential therapies.[15][37][3]



No modifiable risk factor has been identified.[3] Patients can be recommended avoidance of sun and irritating cosmetics as supportive to treatment.[16][18]


Without treatment, MD is unlikely to resolve spontaneously.[26] 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.[1] 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.[1] Oral steroids were correlated with recurrence or progression. Male gender correlated with lack of complete response to treatment.[1] Patients who undergo debulking respond to treatment although response may be partial.[1] Approximately 10% of patients have recurrence or progression of disease.[1]


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