Morbihan Disease Treatment: Two Case Reports and a Systematic Literature Review

Purpose: To assess the effectiveness of treatments for Morbihan disease. Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a systematic review of the literature was performed on April 1, 2018, using PubMed, Google Scholar, and Excerpta Medica dataBASE with terms used to describe Morbihan disease, including "Morbihan Disease," "Morbihan Syndrome," "lymphedema rosacea," and "lymphedematous rosacea". Case reports or case series were included if they fulfilled the following criteria: published in English, peer-reviewed, and reported Morbihan disease. Results: A total of 89 patients—87 patients from 49 articles and 2 cases from the authors' institution—were included in the final analysis. The median age of patients was 51 years (range: 14–79), and 66 of 89 (74%) patients were men. Male gender correlated with lack of complete response to treatment (odds ratio: 0.25; 95% confidence interval: 0.06–0.97; p = 0.02), while presence of papules or pustules correlated with complete response to treatment (odds ratio: 4.07; 95% confidence interval: 1.04–17.68; p = 0.03). Longer antibiotic duration correlated with response to treatment (p = 0.03), favoring complete over partial response (p= 0.02). Mean antibiotic duration in patients who responded was 4.43 months (standard deviation: 3.49), with complete responders requiring 6.50 months (standard deviation: 4.57). Oral corticosteroids, isotretinoins, and combination therapies did not correlate with treatment response. Conclusions: The presence of papules and pustules correlates with a complete response to treatment, while male gender correlates with a partial response. Patients may benefit from 4- to 6-month duration of tetracycline-based antibiotics. Prospective studies are needed to assess the impact of antibiotic and isotretinoin dose and duration on treatment response. Accepted for publication July 31, 2018. This work was partially supported by an unrestricted departmental grant from Research to Prevent Blindness. The authors have no conflicts of interest to disclose. Address correspondence and reprint requests to Gary J. Lelli, Jr, M.D. Department of Ophthalmology, Weill Cornell Medical College, 1305 York Avenue, New York, NY 10021. E-mail: gjl9003@med.cornell.edu © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.