Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. "En coup de sabre" scleroderma is more common, which, by some authors, is treated as variously intensified forms of the same nosologic unit. The etiology of both diseases is not clear .
Frequent coexistence of skin changes within hypodermis and bones of face, with neurological symptoms and with structural and functional changes of CNS are uncontested. Braun – Falco et al.  claim, that in most patients pains within trigeminal nerve innervations occur before visible deformations appear. In case of our first patient, the anamnesis, for many years showed there were weakly intensified, one-sided headaches, treated as migraine. Neurological treatment however, did not show any deviation.
The second patient did not report any subjective symptoms which would accompany skin changes. Neurological treatment also did not show any deviation. CT however, revealed the presence of CNS tumor located subtentorially (in the upper part), towards the posterior side from the callous body and from III cerebral ventricle, without any clear features of growing into these structures.
Aynaci et al.  described a child with hemifacial atrophy features without any neurological deficiency, with Adie's syndrome on the affected side of the face – with mydriasis, no reaction to light, with a slow reaction to convergence and accommodation (tonic pupil). Schnitzler et al.  introduced a 25-year-old patient. At the age of 14, he had a tonic pupil on the right side and epilepsy attacks. At the age of 22, the patient noticed progressive atrophy of the right side of his face. Authors postulate the common, immunological etiology of hemifacial atrophy, Adie's syndrome and epilepsy.
The literature informs that in many patients changes in CNS, visible in CT and NMR appear. Both examinations performed on our patient with diagnosed Perry-Romberg syndrome were normal. The changes described above were found in the patient with „en coup de sabre" scleroderma.
Unterberger et al.  introduced a case of a 24-year-old patient, with „en coup de sabre" scleroderma and with focuses of scleroderma linearis. In the 33rd week of the patient's pregnancy, right hemiplegia symptoms appeared, with a positive Babinski's sign on the affected side. The symptoms escalated significantly after the labor (caesarean section). NMR of the head revealed numerous focuses of an increased signal in the left hemisphere, spreading towards the fronto-temporal area, including the white substance, with minor subcortical hyper intensive focuses from the right frontal side.
In case of the child described by Cory et al. , the symptoms of hemifacial atrophy were developing for a 20 month period. The tomography and the magnetic resonance revealed numerous, one-sided focuses of infarcts in the amygdaloid body, multiple deep and subcortical changes of the signal in the white substance and a mild thinning of the cortex. The angiographic examination was normal. Okumura et al.  performed precise pictorial examination of the patient with Parry-Romberg syndrome. Focuses of an increased condensation in the white substance of the left hemisphere were described in NMR. In the proton spectroscopy, a form of NMR, no aberrations has been found. Monoprotonic, emissive tomography showed an increase of blood perfusion in the left hemisphere. These results correlate with the aberrations in the clinical research and with hemifacial atrophy signs. In the case of a 32-year-old patient described by Moon et al. , with diagnosed Perry-Romberg syndrome, except for the visible face asymmetry, intermittent headaches occurred, accompanied by sensory disturbances affecting the hearing and the sight. NMR revealed a mild asymmetric atrophy of the right hemisphere and few nonspecific white substance condensations on the affected side. DTI (Diffusion tensor imaging) and the tractography confirmed the changes in the nervous fibers of the white substance sensory tracts on the right hemisphere. Sathornsumetee et al.  introduced a case of a 4-year-old boy with a progressive hemifacial atrophy, which was accompanied by multiple neurological symptoms. The patient was diagnosed with epilepsy, progressive hemisphere atrophy and severe changes within the brain stem. Paprocka et al.  introduced a 10-year-old girl patient, with Rasmussen's encephalitis, which is connected with a chronic inflammatory condition and with a damage of one, intact hemisphere. Epilepsy focuses and one-sided neurological symptoms are observed clinically. Right-sided hemiparesis was observed in the patient. From the age of 2, the patient was diagnosed with left hemifacial atrophy and with a focus of „en coup de sabre” linear scleroderma within the forehead. The authors examine the potential relation of linear scleroderma, hemifacial atrophy and described Rasmussen's encephalitis. In 2003 Stone  performed an internet research on 205 patients with Parry-Romberg syndrome and found 11% with epilepsy and 19% with limb motor activity changes. Błaszczyk et al.  examined 19 patients with signs of progressive hemifacial atrophy and en coup de sabre scleroderma towards changes in CNS. Routine examination, EEG, NMR, angio-NMR and 99 mTc-HM-PAO-SPECT were performed. The examinations revealed significantly increased frequency of change occurrence in CNS in patients with hemifacial atrophy and en coup de sabre scleroderma signs than in the control group. Gambichler et al.  introduced a 23-year-old patient with two-sided en coup de sabre scleroderma and with a left-sided hemifacial atrophy. The patient also suffers from epilepsy attacks and a paralysis of oculomotor nerves and of right-sided facial nerve. The authors suggest a close relation between en coup de sabre scleroderma, hemifacial atrophy and neurological symptoms in the patient.
In our first patient examined, antibodies against Borrelia burgdofei in IgM class were found. The infection was confirmed with Western-blot method. It is difficult however, to talk about the relation of a fresh infection with lasting for over 4 years progressive process of facial atrophy. This is rather a coexistence of both diseases. Sommer et al.  research also did not confirm the relation of Borrelia burgdofei infection and the progressive facial atrophy. 278 patients took part in this research.
Relatively few reports concern the treatment of hemifacial atrophy and "en coup de sabre" scleroderma linearis. Most authors use similar medicines as in the treatment of scleroderma: procaine penicillin and other antibiotics, anti-malarial medicine, corticosteroids generally, retinoids, cyclosporine, cyclophosphamide, methotrexat. Locally – emollients, vitamin D analogs, light therapy – PUVA, UVA1 (10–50 J/cm2). This treatment however, aims to speed up the pathological process towards its end, because, in fact there is no medicine for an effective therapy of both scleroderma and Perry-Romberg syndrome. Tollefson et al.  used anti-malarial medicine in 57,1% of patients and methotrexat in 28,6% of patients with hemifacial atrophy and "en coup de sabre" linear scleroderma. The rest of the patients were only taking emollients. The effectiveness in all groups was controversial.
The described patient, without changes in CNS received cefuroxym in a dose of 2 × 1,5 g iv for 10 days, continuing the treatment with oral cefuroxym (Zinnat) for 4 weeks. In the case of the second patient, cefuroxym in a dose of 2 × 1,5 g iv for 10 days was applied and the patient was sent to neurosurgical treatment.
After the pathological process is over, good effects are achieved by plastic surgery, including injection of autologic adipose tissue, synthetic collagen, filling preparation, containing hialuronic acid [1, 5, 15].
These techniques are planned to be implemented in the future.