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

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.