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677 SHORT COMMUNICATION Morbihan Syndrome Successfully Treated with Omalizumab Pegah KAFI, Inger EDÉN and Carl SWARTLING* Kungsholmens Hudklinik, Warfvinges Väg 35, SE-115 21 Stockholm, Sweden. *E-mail: [email protected] Accepted Mar 8, 2019; E-published Mar 8, 2019 Morbihan syndrome (MS) is a rare condition that is considered either a subtype (1) or a late-stage complica- tion of rosacea (2). While its pathogenesis is not fully known, it is characterised by the clinical features of erythema and overt oedema of the upper two-thirds of the face. The erythematous oedema is accompanied by telangiectasias, and occasionally by papules, pustules and nodules. The swelling and distortion in the disease cause visual impairment, discomfort and cosmetic concerns (1, 2). The abundance of mast cells, producing vasoactive substances, proteases and cathelicidins, such as Cath-37, may be important in the pathogenesis of rosacea as well as of MS (3), and studies indicate that the antihistamine ketotifen shows beneficial outcome in the treatment of rosacea (4). Omalizumab is a humanised monoclonal antibody that binds the circulating IgE antibodies and may stabilise mast cells in the treatment of chronic urti- caria (5). Typical flushes in MS and rosacea are similar to the temporary erythematous swellings observed in urticaria and angioedema. Accordingly, we hypothesize that omalizumab could stabilize mast cells in MS and reduce the temporary swelling. CASE REPORT AND RESULTS A 56-year-old woman, with moderate erythematous rosacea, developed persistent and prominent erythematous oedema of the face (Fig. 1A), lasting for one year. Firm swelling of the eyelids, impairing vision, accompanied oedema of the cheeks and forehead. Neither itching nor scaling was observed. Pronounced swelling was seen after exposure to hot, cold and windy weather, and to a lesser extent to stress, sunlight and spicy food. The patient could not work or participate in the social events, due to her symptoms. She was diagnosed with Morbihan disease after excluding aller- gies, various connective tissue diseases such as dermatomyositis and superior vena cava syndrome. A B The patient was tested with a normal skin prick test, tryptase, antinuclear antibodies, thyroid function test, full blood count and X-ray of the lungs and mediastinum. Skin punch biopsy demon- strated non-specific inflammation consistent with rosacea. MS was treated with lymecycline 300 mg twice daily for 3 months and later isotretinoin 10–20 mg daily for 4 months, but there was no effect on the swelling or erythema. Because of the temporary swelling caused by physical stimuli, in addition to the permanent oedema, we initiated a trial treatment with omalizumab 450 mg subcutaneously. A dramatic reduction in the swelling was seen 1–2 weeks post- treatment (Fig. 1B) and the patient subsequently became tolerant to the physical stimuli. The MS cleared completely after 5 months of monthly omalizumab injections, an initial dose of 450 mg and consecutive doses of 300 mg (Fig. 1C). Maintenance treatment was given for a further two months, every 4–6 weeks, and was then discontinued successfully without clinical relapse, 6 months later. The quality of life was markedly improved after omalizumab injections and the patient was able to resume working, instead of being on sick leave. DISCUSSION Our case report explores omalizumab as a therapy option in the treatment of MS, especially when the swelling at- tacks are observed in response to physical stimuli. The patient was completely symptom-free 5 months after the initiation of the monthly omalizumab injections, and 6 months after receiving the last injection she still showed no signs of erythematous oedema or swelling. No side effects were reported. Antihistamines are the first-line treatment for urticaria and angioedema, and omalizumab is the second-line option for the treatment of refractory chronic cases not responsive to antihistamine (6). Omalizumab has been shown to be a potent drug in the treatment of severe ca- ses of urticaria with few side effects (7). Subcutaneous C This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. Fig. 1. A) Pre-treatment photo of prominent and persistent erythematous edema of the face. Firm swelling of the eyelids impairing vision. The patient was on sick leave. B) A reduction of the edema was observed two weeks after the first subcutaneous injection with the monoclonal antibody - omalizumab 450 mg. The patient tolerated windy and cold weather and noticed less tenderness. C) The patient is in her normal condition after monthly injections of omalizumab for five months. Her quality of life was substantially improved, and she was able resume normal life, including returning to work. A written permission from the patient to publish these photos was obtained. doi: 10.2340/00015555-3168 Acta Derm Venereol 2019; 99: 677–678