Diagnosis and Treatment of Morbihan's Disease: A Practical Approach based on Review of the Literature

Abstract

Background: Morbihan's disease (MD), also known as solid persistent facial edema, solid facial lymphedema or rosacea lymphedema, is a rare condition.

Objective: Despite existing case reports and literature reviews, clinical guidance for diagnosis and management is lacking. This review aims to provide comprehensive information on the etiology, differential diagnoses, diagnostics, and management of MD.

Methods: PubMed was searched up to April 2023 for relevant studies on MD with no language restriction. Furthermore, references were checked of found reports.

Results: A comprehensive overview of the literature and clinical guidance for MD. We found 95 studies involving 166 patients (118 male, 46 female and 2 gender unreported) evaluating management options, categorized into: isotretinoin (16 studies), isotretinoin plus antihistamines (8), isotretinoin plus corticosteroids (8), antibiotics (13), antibiotics plus corticosteroids (7), surgical debulking (10) and miscellaneous/combination treatments (43). Some studies contributed to two categories. Treatment with isotretinoin as monotherapy or combined with antihistamines, doxycycline or minocycline as well as surgical procedures demonstrated mostly satisfactory results, although recurrences were common. Longer treatment duration, of at least 6 to 12 months, is recommended for pharmacological treatments. Adding systemic or intralesional corticosteroids to previous treatments may be beneficial. Manual lymph drainage seems to contribute to satisfying result.

Limitations: This is not a systematic review and randomized controlled trials are lacking.

Conclusion: Diagnosis of MD is based on specific clinical features and excluding diseases with similar appearance. Prolonged treatment is often necessary to obtain satisfactory results, which might be limited to a partial and/or temporary response.

Keywords: Morbihan; solid facial lymphedema; solid persistent facial edema.

FIGURE 1.

Various presentations of Morbihan’s disease (consent was given to use unedited images). A) 68-year-old woman with severe erythema and solid edema of the periocular region and cheeks, more pronounced on the left side, with lower lid swelling and festoon and an erythematous plaque on the forehead. B) 49-year-old man with solid periocular edema, more pronounced round the right eye, with slight erythema. C) 59-year-old man showing solid facial edema of the cheeks, glabella, around the eyes and slight facial erythema. D) 72-year-old man with slight erythema and severe solid edema of the right periocular region and cheek with lower lid swelling and festoon. E) 37-year-old woman with erythema and severe solid edema of the left periocular region and cheek, with a few papules on forehead, cheeks and chin. F) The same woman as in Figure 1E, but six years later: recurrence demonstrates erythema and swelling of the cheeks and forehead with papules on forehead and cheeks. Repeated treatments with minocycline 100-200mg for more than three months in combination with oral corticosteroids (tapered) usually resulted in remissions. No recurrences for three years.

FIGURE 2.

A) skin biopsy with the characteristic histology of M. Morbihan (Hematoxylin-Eosin (HE) stained) with mild perifollicular fibrosis and perifollicular lymphohistiocytic inflammatory infiltrate (1), dilated lymphatic vessels (2) and intravascular epithelioid cell granulomas (3). 2B and 2C illustrate an intravascular granuloma: in B, the endothelium of an lymph vessel in the deep dermis is stained by immunohistochemistry using the marker D2-40, in C, the histiocytes of the intravascular epithelioid cell granuloma are stained by an anti-CD68 marker.

FIGURE 3.

Morbihan’s disease over a period of eight years: before and after (consent was given to use unedited images). A) 46-year-old woman with solid edema of periocular regions and cheeks, with slight erythema. B) the same woman seven years later during a recurrence. C) one year later after successful treatment with isotretinoin (20–50mg) for more than six months. No recurrence after six years.