Morbihan’s disease

Abstract

Morbigan’s disease is a rare disease with a progressive recurrent course, which is manifested by swelling of the middle third part of the face and persistent erythema, without objective sensations. This pathology belongs to an atypical variant of rosacea, namely rosacea with solid persistent facial edema. According to modern concepts, in the formation of rosacea, the main role belongs to pathological changes in blood vessels — angiopathies, which develop under the influence of certain endogenous stimuli: vasoactive peptides, antibodies, circulating immune complexes and other substances. Vascular changes are the product of two interdependent pathological processes: violation of the integrity and tone of the vascular wall and disorganization of the perevascular connective tissue, resulting in the formation of a stable expansion of blood vessels of the skin which is clinically manifested by erythema and telangiectasias. Consequently, the constant dilatation of the blood vessels of the skin causes the development of the inflammatory stages of the rosacea process.The etiology and pathogenesis of Morbigan disease are not known; there are assumptions about the role of bacterial infection, hereditary factor, allergy in the occurrence of the pathological process. Diagnostics of Morbigan’s disease is quite complex, in some cases the diagnosis is made only by exclusion. Among the clinical manifestations of the disease, important criteria for the diagnosis are chronic course, absence of changes in laboratory parameters and corresponding histological changes, as well as resistance to therapy. The treatment of Morbigan’s disease has not yet been developed and is a difficult task for dermatovenerologists.The article describes a clinical case of Morbigan’s disease (an atypical form of rosacea with solid persistent edema) in a 63­year­old patient. The presented observation demonstrates the clinical features of the disease. As a result of the successfully selected treatment regimen with the use of isotretinoin, clinical recovery of the patient was achieved.