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.