Solid facial edema in rosacea L71.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 15.12.2022

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Synonym(s)

Facial edema solid in rosacea; Morbihan's disease; Morbihan's disease, rosaceous lymphedema; Oedema érythémateux faciale chronique; Persistent facial swelling in rosacea; rosacea edematosa; Solid persistent facial oedema

History
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Robert Degos, 1957

Definition
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Chronic, persistent, red swelling of the face of unknown etiology accompanied by an unpleasant feeling of tension. No further restriction of the general condition. This clinical picture is probably to be classified as a rare, "oedematous variant" of rosacea.

Occurrence/Epidemiology
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Prevalence is unknown. The clinical picture is often misunderstood or not classified as a special form of rosacea.

Etiopathogenesis
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Like rosacea unknown.

Localization
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Preferably affected are: cheek, nose, eyelids, forehead

Clinical features
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Large-scale, initially inconstant, later persistent, diffuse and blurred, cushion-like swellings and redness of the face associated with an extremely unpleasant feeling of tightness of the skin; rarely also itching.

The swellings are initially succulent soft, later increasingly firm, also coarse and not depressible.

Rosacea may be present at the same time. However, the swelling states do not correlate with the severity of rosacea.

Not infrequently, facial swelling leads to persistent eyelid swelling, which is often considered by patients to be the real problem.

Histology
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The histological picture is not specific. Perivascular and periadnexal lymphocytic and histiocytic infiltrates as well as numerous mast cells are found in the upper and middle dermis. Mast cells are associated with fibrosis.

Histological pattern: Perivascular and peridanexual dermatitis in the upper and middle dermis. Not specific.

Differential diagnosis
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In acute symptoms: systemic lupus erythematosus (the serological systemic symptoms of SLE are absent, serological exclusion).

In chronic persistent swelling: Melkersson-Rosenthal syndrome (with the classic symptom triad: cheilitis granulomatosa, facial paresis, lingua plicata, the distinction is not difficult. More difficult to exclude are the differently pronounced minus variants with recurrent unilateral or bilateral orofacial swellings (eyelid swellings rather speak for the rosacea variant).

Cutaneousmucinosis (plaque-like form of cutaneous mucinosis). Here, 2.0 to 10.0 cm large or larger, single or multiple, red, mostly asymptomatic, moderately indurated, non-scaly plaques are found, which have a punctured, follicle-accentuated surface (peau d`orange). The disease responds to chloroquine as does REM syndrome. chloroquine.

Blepharochalasis: In this "age condition" the inflammatory symptoms and changeability are usually absent. There is an excess of skin without an edematous component.

Therapy
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Pronounced resistance to therapy. Successes are described with isotretinoin (e.g. isotretinoin-ratiopharm; Aknenormin) 0.2-0.5 mg/kg bw/day as monotherapy (Heibel HD et al. 2020) or in combination with the mast cell blocker ketotifen (e.g. Zaditen 1-2 mg/day) over several months.

Alternative: Clofazimine (e.g. Lampren) 4 times/week 100 mg p.o.

Alternative: Thalidomide 100 mg/day.

Alternative: Diuretics - furosemide: 60 mg/day or spironolactone: 75 mg 2x/day(Messikh R et al. 2012) .

Note: The benefit of lymphatic drainage is unclear but widely recommended (Melnik B et al. 2018).

External therapy
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The use of brimonidine can be recommended.

In clinical trials, brimonidine gel had achieved significantly greater improvements in facial flushing in rosacea erythematosa than placebo. Own experience has been favourable! The gel is applied daily in a pea-sized amount to the affected areas. The effect is expected within 30 minutes (Prep. Mirvaso®).

Operative therapie
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In the case of cosmetically disfiguring persistent swelling of the eyelids, the only option left is corrective eyelid surgery. In this indication, the relatively minor surgical intervention should not be delayed.

Note(s)
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The disease is named "Morbus Morbihan" after the region of Morbihan in Brittany, where the disease is apparently more common.

Literature
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  1. Degos R et al (1957) Nouveau cas d'oedème érythémateux faciale chronique. Bull Soc Franc Derm Syph 80: 257
  2. Heibel HD et al (2020) Successful treatment of solid persistent facial edema with isotretinoin and compression therapy. JAAD Case Rep 6:755-757.
  3. Hölzle E et al (1995) Morbihan's disease - Chronic persistent erythema and edema of the face. Dermatologist 46: 796-798
  4. Jansen T et al (1996) Morbihan's disease. Act Dermatol 22: 161-163
  5. Jansen T et al (1998) Persistent erythema and edema of the face associated with rosacea and lymph vessel dysplasia. Dermatologist 49: 932-935
  6. Megahed M et al (2013) Morbihan's disease as a special form of rosacea. Dermatologist 64: 884-886
  7. Melnik B et al (2018) Acne and rosacea. In: Braun-Falco`s Dermatology, Venereology Allergology G. Plewig et al (eds) Springer Verlag p 1330.
  8. Mazzatenta C et al (1997) Solid persistent facial oedema (Morbihan's disease) following rosacea, successfully treated with isotretinoin and ketotifen. Br J Dermatol 137: 1020-1021
  9. Messikh R et al (2012) Efficacy of diuretics in the treatment of Morbihan's disease: three cases.
    Ann Dermatol Venereol 139:559-563 .

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Last updated on: 15.12.2022