Objectives Systemic sclerosis (SSc) is one of the few somatic disorders which induce isolated or combined trigeminal sensory neuropathy (TSN).

Methods A screening of 101 patients with SSc hospitalised at the Institute of Rheumatology over a period of six months in 2000 revealed that eight patients (7,9%) had TSN (all of them were females aged 42–69 with a clinical history of SSc of 1 to 12 years). In two cases, SSc was associated with the SJÖGREN’S syndrome; one case was associated with polymyositis. All the patients with TSN underwent rheumatological and neurological examinations including the recording of blink reflex.

Results The main clinical manifestation of TSN was bilateral asymmetric numbness in all the three divisions of the trigeminal nerve, especially in the perioral area. Five patients had qualitative taste disorders including «oral astereognosis», i.e., monotypical sensations in the oral cavity on taking up foods of different kinds and tastes. In two cases, TSN was manifested as constant and constant or «neuralgia-like» facial pains. Irrespective of remissions (which could be spontaneous), in each case facial pain was one of the most significant manifestations of SSc. All the patients had disturbances in surface sensitivity (hyper- or hypoesthesia) in the distal parts of the upper and lower extremities. In three cases, early symptomes of TSN (e.g., facial paraesthesia, numbness and hyperesthesia) developed simultaneously with the changes in the skin colour (especially of fingers), skin oedema, arthralgias and malaise. In the rest of the patients, TSN developed within 6 months to 3 years after the Raynaud’s syndrome, sclerodactyly and other manifestations of SSc. The blink reflex records revealed modest prolongation of ipsilateral R1 responses. Specific therapy of TSN included administration of vasoactive drugs (e.g., pentoxifylline, vazaprostan) and group B vitamins. The elimination of facial pain presented a problem. The use of NSAID resulted in short-term attenuation of pain; in one case, topical application of ibuprofen (in the form of a cream) was more effective. Patients with paroxysmal manifestations of pain received additionally carbamazepine (300–400 mg/d). The efficiency of physio- and reflexotherapy in patients with acute facial pain syndrome is open to question.

Conclusion TSN is a typical neurological sign of SSc (with frequency 7,9% of cases), which is manifested as facial numbness, qualitative taste disorders including «oral astereognosis», pain and paraesthesiae. Most often, TSN develops within 6 months to 3 years after the appearance of other characteristic features of SSc. A combination of bilateral sensory disturbances in the facial area and distal parts of the upper and lower extremities suggests that TSN can be regarded as a partial form of generalised sensory polyneuropathy.