Fuchs syndrome

Fuchs syndrome is a minus variant of erythema multiforme majus, defined as inflammatory involvement of two or more mucosal surfaces without the characteristic skin involvement of EM such as typical (iris lesions) or atypical cockades.

Fuchs syndrome is mainly associated with M. pneumoniae infections.

Oral lesions with mucositis are most frequently observed, ocular involvement (conjunctivitis) occurs in around 67% of cases and genital mucosal involvement in around 75% of cases. Erythematous, erosive and ulcerative lesions are found.

The mucosal lesions of Fuchs syndrome require a rapid and appropriate diagnosis due to the sudden onset of their painful symptoms, followed by immediate treatment. Oral lesions can lead to malnutrition or disturbances of fluid and electrolyte balance, while conjunctival lesions can cause symblepharon, keratoconjunctivitis sicca, ectropion or corneal opacities, among others.

Treatment is otherwise supportive; macrolide antibiotics are the treatment of choice for children.

With timely treatment, the prognosis is excellent.

A 29-year-old man was referred to the Department of Dentistry by his GP because of an acute eruption on his oral mucosa that prevented him from eating. The lesions had appeared 8 days earlier and prior to this the patient had had a low-grade fever, headache and sore throat. He had been prescribed non-steroidal anti-inflammatory drugs and cetylpyridinium lysozyme for treatment. The patient also reported that he had suffered from several episodes of cold sores prior to the onset of symptoms.

General symptoms such as asthenia and fever were observed. The initial cardiopulmonary examination was normal. There was no swelling of the lymph nodes, liver or spleen. Oral examination revealed multiple but irregular oval erosions on a reddened surface on the palate, inner cheeks and soft palate arches. His tongue was swollen with lateral impressions of the teeth. Ulceration of the orificium urethrae and bilateral conjunctivitis were also noted. His vision was not impaired. Serology for M. pneumoniae was positive. However, the polymerase chain reaction for herpes simplex virus was negative. The C-reactive protein reached 54 mg/l.

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