Rosacea Lymphedema (Morbihan Disease)

[Guide]

The RRDi recognizes Rosacea Lymphedema  (Morbihan Disease [Syndrome] or Morbus Morbihan) as a rosacea variant. [1] Also known as rosacea lymphoedematous or persistent solid facial edema [2], Persistent edema of rosacea or Chronic upper facial erythematous edema. [3] "In the cases presented, initial clinician suspicion for Morbihan syndrome was high given the past medical history of rosacea in each patient." [9]

Images of Rosacea Lymphedema. 

"Morbihan disease (MD) is a rare entity. Its nosography is unclear and its therapeutic management is difficult....the patient was put on isotretinoin and furosemide with slight improvement. The particularity of our observation lies in the rarity and especially in the therapeutic difficulties encountered during this disease.  [4]

It is also known as Morbihan syndrome, "a rare entity that more commonly affects women in the third or fourth decade of life." [4] "Morbihan syndrome is a rare and chronic condition. It can be difficult to treat and may require a range of interventions." [5]

Morbihan diseases should be differentiated from Melkersson-Rosenthal syndrome. [6]

More often than not, Rosacea Lymphedema is referred to as Morbihan Disease, and is often recalcitrant to therapy, is a cosmetically disturbing condition, and unfortunately mostly refractory to the therapeutic measures listed below. 

"Morbihan syndrome is a rare disease of unknown etiology presenting with erythema and edema of the upper two-thirds of the face." [9]

"MD often tends to be unresponsive to therapies commonly used to treat rosacea, including corticosteroids, isotretinoin, and antibiotics." [10]

Treatments

"2.5% hydrocortisone cream, brimonidine 0.33% topical gel, metronidazole gel and 100 mg doxycycline twice daily." [9]

CO2 laser blepharoplasty

Complete Decongestive Therapy (CDT)

Ketotifen and isotrenioin [8]

Omalizumab

"Reported therapy includes short-term oral isotretinoin (0.5 mg/kg/day), long-term oral isotretinoin (40-80 mg/day, 10-24 months), long-term doxycycline, combination of systemic corticosteroids and antibiotic (prednisolone 20 mg/day for 2 weeks and doxycycline 200 mg/day for 12 weeks), slow-releasing doxycycline monohydrate (40 mg/day for 6 months), long-term minocycline (50 mg/day for 4 months), and a combination of both oral retinoid and ketotifen (isotretinoin 0.7 mg/kg/day for 4 months, ketotifen 2 mg/day for 4 months).

Surgical Lymphoedema Treatment

Tripterygium wilfordii 

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End Notes 

[1] "Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known..."

An Bras Dermatol. 2016 Sep-Oct; 91(5 Suppl 1): 157–159.
doi:  10.1590/abd1806-4841.20164291
PMCID: PMC5325027
Morbihan syndrome: a case report and literature review
Rossana Cantanhede Farias de Vasconcelos, Natália Trefiglio Eid, Renata Trefiglio Eid, Fabíolla Sih Moriya, Bruna Backsmann Braga, and Alexandre Ozores Michalany

"Morbihan disease, also known as rosacea lymphedema, is a rare persistent form of lymphedema that is associated with the disease of rosacea."

Phys Ther. 2018 Dec 18;
Complete Decongestive Therapy Is an Option for the Treatment of Rosacea Lymphedema (Morbihan Disease): Two Cases.
Kutlay S, Ozdemir EC, Pala Z, Ozen S, Sanli H

[2] "Morbihan disease is a rare entity. Its place in the nosography is uncertain. Sometimes called persistent solid facial edema, sometimes rosacea lymphoedematous. It may correspond to a particular clinico-pathological form of lymphoedema or rosacea."

Pan Afr Med J. 2018; 30: 226.
Published online 2018 Jul 26. doi: 10.11604/pamj.2018.30.226.14440
PMCID: PMC6295304; PMID: 30574244
Morbihan disease: treatment difficulties and diagnosis: a case report
Alaa Aboutaam,& Fouzia Hali, Kenza Baline, Meryem Regragui, Farida Marnissi, and Soumiya Chiheb

[3] Persistent edema of rosacea, Wikpedia

[4] Pan Afr Med J. 2018 Jul 26;30:226. doi: 10.11604/pamj.2018.30.226.14440. eCollection 2018.
Morbihan disease: treatment difficulties and diagnosis: a case report.
Aboutaam A, Hali F, Baline K, Regragui M, Marnissi F, Chiheb S.

[4] Morbihan syndrome

[5] Ophthalmic Plast Reconstr Surg. 2020 Jan 23;:
Morbihan Syndrome, a UK Case Series.
Yvon C, Mudhar HS, Fayers T, Siah WF, Malhotra R, Currie Z, Tan J, Rajak S

[6] Dermatol Online J. 2020 Jun 15;26(6):
Morbihan disease: a case report and differentiation from Melkersson-Rosenthal syndrome.
Kuraitis D, Coscarart A, Williams L, Wang A

[7] Acta Dermatovenerol Croat. 2020 Aug;28(2):118-119
Morbihan Disease - An Old and Rare Entity Still Difficult to Treat.
Jerković Gulin S, Ljubojević Hadžavdić S

[8] Solid persistent facial oedema (Morbihan's disease) following rosacea, successfully treated with isotretinoin and ketotifen. (1997)
Br J Dermatol. 1997 Dec; 137(6):1020-1.

Solid persistent facial edema of acne: successful treatment with isotretinoin and ketotifen
B Jungfer  1 , T Jansen, B Przybilla, G Plewig PubMed (1993)

[9] SAGE Open Med Case Rep. 2021; 9: 2050313X211023655.
Morbihan disease: A diagnostic dilemma: two cases with successful resolution
Deepak Donthi, Joseph Nenow, Arthur Samia, Charles Phillips, John Papalas, Karyn Prenshaw

[10] Treatment of Morbihan disease

[Guide]

"Morbihan syndrome is characterised clinically by the slow appearance of erythematous and solid, non-pitting oedema localised exclusively on the forehead, glabella, eyelids and cheeks. The aetiopathogenesis of Morbihan syndrome is not completely known. Most authors propose that it is a clinical variety or a complication of acne or rosacea. Some authors hypothesise that Morbihan syndrome may be caused by lymph vessel abnormalities.6 Pathological examination, although non-specific, is characterised by perivascular dermal oedema with a lympho-histiocytic periadenexal infiltrate containing numerous mast cells and dilation of lymphatic vessels. Granulomas are sometimes present, and sebaceous gland hyperplasia can be observed in patients who have had or have associated rosacea.

The age, clinical picture (erythema, pustules and telangiectasia on the face) and histological features consistent with granulomatous rosacea (marked oedema and granulomas) are all supportive of the diagnosis of rosacea leading to Morbihan syndrome.

According to the current literature, treatment is challenging. Commonly used treatments are tetracycline group antibiotics and isotretinoin. Other treatment modalities include facial massage to improve drainage and steroids. Isotretinoin (10–20 mg/day) alone or in combination with ketotifen (1 mg two times per day) for 3–6 months has been reported to be effective. However, the response is unpredictable."

BMJ Case Rep. 2019; 12(10): e231074.
Rosacea causing unilateral Morbihan syndrome
Saliya Weeraman and Andrew Birnie