Melkersson–Rosenthal syndrome. Clinical case

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Abstract

This clinical case is of interest due to the rare occurrence of Melkersson–Rosenthal syndrome in the population, as well as the difficulty in diagnosing and achieving remission of this disease.

Melkersson–Rosenthal syndrome is a rare chronic relapsing dermatosis in the population, in typical cases characterized by a triad of symptoms: macrocheilitis, neuropathy of the facial nerve and folding of the tongue. However, much more often the disease manifests monosymptomically. The etiology of the syndrome has not been definitively studied: the infectious and allergic component, inflammatory processes of the facial nerve, genetic predisposition, as well as contact with metals (cobalt and gold) are considered as the determining causes. The diagnosis is based on clinical data and the results of a pathomorphological study. With a monosymptomatic (only the presence of macroheilitis) course of Melkersson–Rosenthal syndrome, a differential diagnosis is carried out with angioedema Quincke, lymphangioma, hemangioma, lip abscess and other cheilitis.

The clinical picture of dermatosis is localized on the face and is a significant problem, significantly affecting the social status of patients and is highly threatened in relation to the development of dysmorphophobia.

The presented clinical case is rare in the practice of a dermatologist, patients with a similar pathology turn to dentists, allergists, where the clinical picture is regarded as Quincke’s edema. Given the rare occurrence of Melkersson–Rosenthal syndrome in the population and the difficulty of diagnosing the disease, the presented clinical case is indicative, since in our case the patient has only 2 main symptoms (folded tongue, macrocheilitis) and there is no clinical picture of facial neuritis.

Timely diagnosis and a thorough examination of patients with Melkersson–Rosenthal syndrome contribute to the greatest effectiveness of therapy and the achievement of a stable remission of the disease.

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About the authors

Elena S. Snarskaya

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: snarskaya-dok@mail.ru
ORCID iD: 0000-0002-7968-7663
SPIN-code: 3785-7859

MD, Dr. Sci. (Med.), Professor

Russian Federation, 2, building 2, Bolshaya Pirogovskaya st., Moscow, 119992

Yana N. Medvedeva

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: dr.yana.med@bk.ru
ORCID iD: 0000-0003-2323-4339

MD

Russian Federation, Moscow

References

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Supplementary files

Supplementary Files
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1. Fig. 1. Patient V., 36 years old, Melkersson-Rosenthal syndrome: pronounced persistent swelling of the upper lip of a bright pink color. Asymmetry in the area of the nasolabial triangle.

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2. Fig. 2. The same patient: а ― tongue folding with unevenly pronounced foci of epithelial desquamation, papillary hypertrophy; b ― swelling of the upper lip and tongue in lateral projection.

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