a 48-year - old female with complaints of prominence of left forehead and asymmetric left - sided hemifacial atrophy , alopecia , asymmetric thinning of left lower leg , without any history of trauma , presented to the dermatology department of our hospital .
she also had slight skin pigmentation over the left eyebrow and the left palpebral conjunctiva .
her symptoms started around 6 years ago with mild hemifacial atrophy on the left forehead , and progressed slowly with alopecia .
the asymmetric thinning of her left lower leg had started around one year before . no facial motor and sensory deficits were noted physically with normal tongue movements , without any enophthalmus or ptosis of the left upper eyelid .
the left - sided facial and forehead asymmetry was detected mainly due to subcutaneous fatty loss with prominent bony ridges on the left forehead and left maxillary region .
intra and extraoral examination revealed normal physical yields without any atrophy of chewing muscles and/or tongue .
paranasal sinus x - ray examination also presented a normal frontal and maxillary sinus on the left side .
there was asymmetric prominent subcutaneous fat and tissue loss in the left cruris region on inspection ( figures 1a & 1b , figures 2a & 2b ) .
she had several normal brain ct scans and electromyographic approaches during the first 6 years .
following consultation , she was admitted as inpatient admission to the surgery department , then referred to egerad private medical imaging center for brain mri .
the brain mri revealed asymmetric , prominent fatty loss in the dural and galeal parts of the left frontal region .
no cortex or deep white matter involvement , including white matter hyperintensities - cortical atrophy was indicated ( figures 3a - c ) .
an image showing : a ) general physical appearance of the patient ; and b ) left sided facial hemiatrophy and left forehead prominence with bony ridges .
an image of the patient showing ; a ) asymmetric fatty loss and thinning of left lower leg ; and b ) non - homogenous alopecia .
an image showing : a ) fatty loss of the scalp and galeal subcutaneous fatty tissue of the left frontal area , seen in t2 weighted ( w ) axial image ; b ) tissue loss of the scalp of the left frontal region , presented in t2w coronal image ; and c ) calvarial fat and soft tissue loss in left frontal side , shown in t1w axial image .
laboratory results for a complete blood count and biochemistry revealed hemoglobin : 13.58 [ normal value ( nv ) : 12 - 16 g / dl ] , hematocrit : 41.3 [ nv : 37 - 50 g / dl ] , mean corpuscular volume : 90.2 [ nv : 80 - 99 g / dl ] , mean corpuscular hemoglobin 28.5 [ nv : 33 - 37 g / dl ] , white blood count ( 4700/lt [ nv : 4800 - 10800/lt ] , c - reactive protein 2.23 mg / dl [ nv : 0 - 10 mg / dl ] , sedimentation 38/hour [ nv : 0 - 20/hour ] , and mild thrombocytosis ( 430.000 g / dl ) were indicated .
prothrombin time , and activated partial thromboplastin time were normal with negative hepatitis b surface antigen , anti hepatitis b surface antibodies , hepatitis c virus , and human immunodeficiency virus .
urine analysis was normal , csf analysis showed mildly elevated protein ( 16 mg / dl ; nv : 0 - 10 mg / dl ) , normal glucose and no pleocytosis . in the differential diagnosis , scleroderma or connective tissue disorders were considered , however , she had negative hla - b27 with normal glucose and electrolyte levels in serum blood .
based on the clinical findings and history , a diagnosis of parry - romberg syndrome was made .
alloplastic aesthetic surgery with autologous fat injection to the atrophied left forehead was suggested to our patient by a plastic surgeon , but due to financial difficulties , she declined any surgical management .
the treatment plan excluding the surgical management of our patient was physical and clinical follow - up in our research hospital ; the follow up included physical rehabilitation including sports and exercise , use of a chin mask , and/or analgesic use .
it is characterized by a slow progressive unilateral atrophy of the face , including skin - soft tissue , muscles and bones , which were present in our case.1 - 5,9 the extension of the atrophy is frequently limited to one side of the face , and ipsilateral facial involvement is rare ( 10 - 20% of cases were reported bilaterally).2 - 5,8,9 the early hemifacial asymmetry progresses over years to extreme loss of subcutaneous tissues , resulting in a sunken and wrinkled face on one side.5,8,9 the incidence of prs is not well - established , the average onset of disease is around 10 years of age ; however , onset can be found as late as 40 - 50 years in some patients;8 - 10 our patient was a 48-year - old female .
the severity of prs with late onset , including facial atrophy - asymmetry , neurological and ocular findings and so forth , may be less than prs in younger ages , probably due to the complete development of craniofacial and nervous structures.5,8 - 10 to date around 150 cases of prs had been reported in the literature.1 - 10 this syndrome is more prevalent in females without any precise geographical distribution .
it mostly manifests on the left side of the face , and can occur in any members of the same family.1 - 5,8 characteristically , hemifacial atrophy is usually self - limited and stabilizes in most of the patients after 5 - 10 years duration.8 - 10 guerrerosantos et al10 classified prs into 4 types : type 1 and 2 involve a decrease in the facial soft tissue , type 3 and 4 also involve thinning of soft tissue , as well as bone and cartilage atrophy .
type 1 is the mildest form , and type 4 is the most severe , and most serious form.10 our patient is considered to be type 3 due to thinning of the subcutaneous soft tissue in the left face and cruris region .
electromyography , blink reflex , and trigeminal evoked potential abnormalities may indicate false implications through the brain stem as the etiology of the disease , and hyperactivity of the brain stem sympathetic centers , which may be caused by autoimmune processes , may lead to the cutaneous and subcutaneous atrophy in prs.1,5,10 clinically , dry and hyperpigmented skin may be the first sign , also present in our patient , and in some cases , a demarcated line between normal and abnormal skin can be seen , known as the en coup de sabre that means
 cut of the sword , can also be shown in a localized form of scleroderma.1 - 3 ocular involvement is common , such as progressive enophthalmos , pseudoptosis , uveitis , pupillary disturbances , heterochromia , and restrictive strabismus .
the most frequent opthalmic manifestation is enophthalmos due to loss of fat around the affected orbit , which was not observed in our patient.2 - 5,9 alopecia and thinning of the ear due to fat atrophy on the involved side are also frequent signs ; alopecia was present in our case.1,9,10 neurological complications of prs are trigeminal neuralgia , facial paresthesia , migraine , and seizures .
epilepsy , especially contralateral epilepsy may also occur occasionally,5,10 however , except for headache , other findings were not found in our patient .
atrophy of the tongue on the affected side , deviation of the mouth and nose to the affected side , atrophy of the upper lip leading to an exposition of teeth , small teeth with short roots on the involved side are the other important features of prs , which were absent in our patient.8 - 10 although the pathophysiology of this disease is not well known , some authors have proposed that the primary cause is neurologic disturbance of fat metabolism , resulting from a trophic malformation of the cervical sympathetic nervous system.5,9,10 parry - romberg syndrome may have strong correlation with autoimmune disorders , endocrine , and hereditary disturbances , scleroderma , and rasmussen encephalitis.1,5,10 neuroimaging findings of prs may be intracranial calcifications , cerebral and cerebellar atrophy , deep white matter lesions , encephalomalacia , heterogeneous meningeal enhancement and so forth.5,10 the brain mr imaging of our patient showed asymmetric , prominent fatty loss in the dura and galea of the left frontal side without any deep white matter and cortical involvement of the cerebrum and cerebellum .
there is no curative therapy for prs and treatment options are limited , symptomatic treatment including pain relief , anticonvulsant drugs , and reconstructive surgery are the main management modalities ; steroid and immunosuppressive therapy have also been tried in some cases.3,10 surgical treatment is usually based on the reposition of fat on the atrophied side , autogenous fat and cartilage grafts , silicon injections and prostheses , bovine collagen and inorganic implants are the alternatives of aesthetic surgical correction of fatty atrophy.3,9,10 our case had left face asymmetry with slight left facial hemiatrophy , a prominent left forehead with bony ridges due to subcutaneous fatty tissue loss , slight pigmentation over the left eyebrow and conjunctiva , and alopecia , which are the classical features of prs in previous reports.1 - 10 she had normal neurologic and ocular findings , with normal psychomotor functions , and brain mri revealed an asymmetric fatty loss in the left frontal side without any cortex or deep white matter involvement .
she also had subcutaneous fatty loss in the left lower leg , resulting in an asymmetric thinning of the left cruris , which on literature review , is a previously unknown feature of prs.1 - 10 due to the non - existence of a precise cure , affected patients should be managed by a multidisciplinary team of physicians , surgeons , dentists , and psychologists to alleviate all possible difficulties related to prs . fat and dermis grafts are the most appropriate for type 1 and 2 prs , whereas panfacial volumization with autologous fat - cartilage and bone graft - galeal flap injection is an excellent tool for replacing volume and restoring contour to the aging face in type 3 and 4 prs.3,10 alloplastic aesthetic surgical implantation with lipoinjection was suggested for our patient , but she declined . in conclusion , prs is a rare disease that manifests with facial hemiatrophy and subcutaneous fatty loss on the affected side ; its pathophysiology remains unknown .
no definite curative treatment exists for prs , and the most widely available treatment approach is based upon plastic aesthetic surgery .
 progressive hemifacial atrophy also known as parry - romberg syndrome is an acquired , slowly progressive disorder , occurring more in women , primarily affecting one side of the face , mainly characterized by unilateral atrophy , and loss of skin and subcutaneous tissues of face , muscles , and bones . 
 ocular and neurologic involvements are common . 
 the possible etiology is unclear without any known cure . 
 we report a rare case of parry - romberg syndrome with classical features . 
 the clinical features , radiological imaging findings , differential diagnosis , and available treatment options are discussed in this report . 
Case Report
Discussion