Morbihan disease is a rare condition, manifested centrofacial, solid, permanent edema, accompanied by multiple telangiectasias, papules-pustular elements, with a primary lesion of the middle 1/3 of the face. Typical is a dark red edema with a purple tint, turning into induration. Subjective symptoms are usually absent, except in cases of complete closure of the eye slit. The pathogenesis of the disease is not well understood. There is a view that the Morbihan disease is a severe manifestations of rosacea with a part in the pathogenesis of vasoactive peptides in the gastrointestinal tract (VIP, pentagastrin), causing tides. Provoked by alcohol tide reactions associated with lack of enzymatic destruction of alcohol, particularly in individuals of the Eastern nationalities. At the pathogenesis of the disease also plays a role in increasing the level of mediator substances (endorphins, bradykinin, substance P), which change the tone of blood vessels, increase permeability, causing characteristic vascular changes and mast cells, which important in the development of edema. Other authors argue that the disease is a consequence of abnormalities in the lymphatic vessels of the face. It is believed that the basis of the pathological process in this disease is the destruction of the connective tissue of the perivascular dermis, especially elastin, which leads to a loss of integrity of the walls of blood vessels in the release of fluid that causes swelling and compression of granulomas of the vessels of the face. There are data that have demonstrated the presence of an immunological component in some patients by the type of urticaria in combination with local lymphatic disorders.
Clinically the disease is characterized by a slowly growing dense erythema and edema in the middle part of the face, with accentuation in the periorbital region, the lower 1/3 of the forehead, nose and cheeks. Laboratory results are non-specific. Histological picture reveals atrophy of the epidermis, discrete edema of the middle and deep dermis, inflammatory infiltrates consisting of lymphocytes, histiocytes and neutrophils in the perivascular and perifollicular areas, superficial fibrosis. Differential diagnosis is carried out with orofacial granulomatosis, sarcoidosis, Hansen’s disease, lupus erythematosus. Such changes may occur as a result of side effects with long-term use of barbiturates, chlorpromazine, diltiazem.
Currently there are no uniform standards for the treatment of Morbihan disease. Therapeutic schemes using lymphatic massage, drugs, thalidomide and high doses of broad-spectrum antibiotics (doxycycline, minocycline), oral prednisolone and metronidazole with little effect are offered.
The report presents a case of successful treatment of the disease Morbihan from the author’s personal archive with photos before, during and after treatment. The patient received systemic isotretinoin Lidose from the calculation of 0.3 mg/kg/day for 7 months, then - maintenance therapy for 16 mg 2 times a week for 2 months, then 8 mg 2 times a week for 1 month. At the same time, the patient received ketotifen 2 mg/day for 10 months. Local treatment – cream with ivermectin 1% - 1 every day for 5 months, then – gel based on azelaic acid 1 once a day a month. As a result of the treatment, the patient almost completely resolved swelling and hyperemia of the face, periorbital induration, there was a complete opening of the eye cracks, decreased the number of telangiectasia, resolved papular pustular elements, greatly improved the quality of life of the patient. Observation of the patient within 2 months after the end of therapy showed no signs of relapse.