What are the features of the course of Morbigan's disease?

In 1957, a rare disease of the skin of the upper half of the face was first described, which is characterized by swelling and erythema. Morbigan's disease got its name from the French region where the first patient suffering from dermatosis of unknown etiology lived.

The disease is characterized by persistent dense asymmetric swelling of the bridge of the nose, upper eyelids, nose, cheeks. Due to swelling of the eyelids, partial ptosis may form. The revealed violation of microcirculation in this disease is close to the changes in rosacea. Find out the symptoms, diagnosis and treatment of Morbigan's disease at estet-portal.com.

Features and differential diagnosis of Morbigan's disease

At first, the disease proceeds chronically with periodic exacerbations, then it becomes persistent, the severity of symptoms increases.

The etiology and pathogenesis are unknown, there are suggestions about the role of a bacterial infection, a hereditary factor, an allergy in the occurrence of a pathological process.

Clinical and biochemical tests in people with this rare disease are usually within normal limits. A clinical case of Morbigan's disease, read more on estet-portal.com. Diagnosis of Morbigan's disease is difficult.

The differential diagnosis of Morbigan's disease should be made with:

• Allergodermatoses (allergic dermatitis, urticaria);
Quincke's edema
• ; Connective tissue diseases (
systemic lupus erythematosus
• , dermatomyositis);
Rosacea disease
• , sarcoidosis, erysipelas, leishmaniasis, lymphoma, Merkelson-Rosenthal syndrome. Histological studies in the skin reveal dermal edema, dilation of the lymphatic vessels of the skin, perivascular and perifollicular histiocytic infiltrate, an increase in the number of mast cells, fibrosis of the dermis.
Description of a rare clinical case of Morbigan's disease

A case is described in which a patient was diagnosed with rosacea with solid persistent edema (Morbigan's disease). This patient was followed up for 5 years.

Of the clinical manifestations, the patient had a regional rash with primary elements of the rash in the form of spots and papules, which were localized on the forehead, eyelids, eyebrows and bridge of the nose.

On palpation, the edema did not leave a hole. There was swelling of the upper eyelids. The results of the histopathological conclusion - the dermis is edematous, there are separate lymphohistiocytic infiltrates with perivascular localization and hyperplasia of the sebaceous glands.

No cure has been developed because the disease is rare and every case reported in the literature is difficult to diagnose. Treatment for Morbigan's disease was symptomatic and often ineffective.