Discussion
PRS is a rare disease with a female predilection and ratio of 1:70,000
and is mostly seen in the left half of the face. The prevalence of PRS
in boys is lower and its ratio is 1:38. It is believed that
brain disorders in fat metabolism, local facial trauma, endocrine
disorders, autoimmunity, heredity, decreased or increased sympathetic
nervous system activity, trigeminal nerve abnormalities and viral
infections are related to the pathogenesis of PRS9. PRS has been
associated with developmental and congenital abnormalities, such as
neurological, ocular, cardiac, cranial, jaw, and facial abnormalities.
Stress (26%) and surgery (8%) have also been identified as possible
triggers for the acceleration of the disease, which usually involves the
lower part of the face, and deeper involvement of bones, teeth, tongue,
and gums may also be seen10. It should be noted
that this involvement is without significant epidermal change4, although PRS usually
refers to the atrophy of dermatomes of one or more branches of the fifth
cranial nerve 2, and in
general, skeletal hypoplasia of the affected areas of the skull is also
common 11. In the
presented case, skeletal hypoplasia was not seen in the skull and only
the fat and subcutaneous tissue had atrophied. Positive ANA is the most
common laboratory abnormality and approximately 25-52% of PRS patients
have high antibody titers8. However, no positive
antinuclear antibody was reported in the tests of this patient.
Rheumatoid factor (RF) along with local scleroderma and extra cutaneous
involvement has been shown in arthritis patients. However, it remains
unclear whether any PRS patients regularly have elevated RF12. In imaging
findings, ultrasound can be used to detect the presence of sclerosis and
monitor the progress of the disease and the progress of treatment by
measuring the thickness of the skin and the echogenicity of the affected
areas. Color Doppler ultrasound has the additional advantage of
measuring skin blood flow, the increase of which indicates active
disease 13. Considering
the frequency of neurological complications in PRS, basic imaging
(CT-scan, MRI), especially in patients with neurological symptoms, can
be performed 8.
The timing of surgical intervention in patients with PRS has been
discussed. Most experts recommend that treatment be delayed until the
progression of the disease stops or gradually stops, so that the
surgical site can achieve a stable skeletal base along with the
progression of the defects and multiple surgeries can be avoided. For
more severe atrophy, a combined approach of strengthening both skeletal
and soft tissue is recommended. Bone paste cranioplasty, skin fat
grafting, and facial fat flaps can also be used to correct large volume
atrophy. Brow lift, Z-plasty, lip reconstruction, nose reconstruction,
eyebrow reconstruction, face lift, lip augmentation, hair transplant and
other auxiliary procedures can also be used to create a better cosmetic
result 14-16. However,
in addition to aesthetic concerns, this syndrome creates functional and
psychological problems for patients, which requires a multidisciplinary
team approach to identify the treatment expectations of these patients17.