Parry–Romberg syndrome in an adolescent: a case report on progressive hemifacial atrophy

Srinivasan, Ranjini; Joy, Shasta Theresa

doi:10.1093/omcr/omz127

Cited by 6 publications

(4 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Similar clinical and histopathological findings suggest that PRS and ECDS, although representing different clinical entities, lie on the same disease spectrum, and the overlap is described in 28-42% of cases [9]. Clinically, ECDS can present with band-like changes of sclerosis and hyperpigmentation with induration, and PRS with unilateral atrophy without induration or inflammation [8].…”

Section: Discussionmentioning

confidence: 66%

See 1 more Smart Citation

Parry Romberg Syndrome: When the Diagnosis of a Rare Disease Is Made in the Primary Care Setting

Pereira,

Rodrigues,

Nunes

et al. 2023

Cureus

View full text Add to dashboard Cite

Parry Romberg syndrome (PRS) is an acquired neurocutaneous syndrome with uncertain pathophysiology, and its incidence is unknown. Usually, the disease becomes apparent during the first decade of life or early during the second decade, but it can also occur in adulthood, and it is more common in females. The main feature is slowly progressive hemiatrophy (thinning or shrinkage) of the facial tissues, typically fat, skin, connective tissues, muscle, and sometimes bone. In some people, atrophy may also affect the trunk and the limbs. Additional symptoms can potentially develop in some patients, including ophthalmological, psychiatric, and neurological complications. The clinical presentation serves as a guide for the diagnosis. Treatment can demand a multidisciplinary approach (maxillofacial surgeons, plastic surgeons, ophthalmologists, neurologists, dermatologists, psychiatrists, anesthetists, and family doctors). Patients can undergo restorative plastic surgery to improve their appearance, with highly variable success rates. We present a case report of a 52-year-old man who made an appointment at the family care unit (FCU) because of a left facial hemiatrophy that started progressing two to three months before, and he was afraid it might be cancer. At the physical exam, it was possible to examine a slight hemiatrophy in two different parts of the left side of the patient's face (the nasolabial-masseter region and the temporal-malar region). The facial CT scan showed a low degree of maxillary bone resorption.Through discussion with peers on the Family Doctor team, the diagnosis of a rare condition in the primary care setting was made possible.This case shows the importance of being aware of a rare disease despite working as a family physician and aims to make more people familiar with this syndrome. It also raises awareness about the need for discussion of clinical cases as a team.

show abstract

Section: Discussionmentioning

confidence: 66%

“…Diagnosis can be based on characteristic clinical findings, detailed patient history, and clinical evaluation without the need for further investigation [8]. Differential diagnoses include hemifacial microsomia, Bell's palsy, lipodystrophies, and a form of linear scleroderma on the scalp and forehead termed "en coup de sabre" (ECDS) [1,5].…”

Section: Discussionmentioning

confidence: 99%

Parry Romberg Syndrome: When the Diagnosis of a Rare Disease Is Made in the Primary Care Setting

Pereira,

Rodrigues,

Nunes

et al. 2023

Cureus

View full text Add to dashboard Cite

show abstract

“…Parry Romberg Syndrome is a rare disorder with incidence of 1:70000 and it has a female predilection. 4 Mostly it affects the left side of the face and starts in second decade of life. Progression of the disease ranges from two to ten years and after that disease may cease itself.…”

Section: Discussionmentioning

confidence: 99%

Parry Romberg Syndrome: a case report

Liaquat

Riaz²,

Shah³

2020

JCMC

View full text Add to dashboard Cite

Parry Romberg syndrome (PRS) is a rare disorder, characterized by unilateral facial atrophy of the skin, subcutaneous fat, muscles, cartilages, and even bone. Various systemic manifestations reported with this syndrome are neurologic, maxillofacial, and ophthalmologic. The etiology is still unknown. Herein we present a case report of 23 years young engineering student who had PRS and got a fracture of the right angle of his mandible after a road traffic accident. The treatment was challenging as he had nonunion at the fracture site.

show abstract

“…U prikazanom slučaju, primećena je linija koja razgraničava normalnu kožu od abnormalne kože, koja je označena kao "presek sabljom", slična linearnoj sklerodermiji. PRS i linearna skleroderma pripadaju istoj grupaciji bolesti i oko 28% -42% slučajeva PRS-a koegzistira sa lokalizovanom sklerodermom 8 . Segna i saradnici 9 prijavili su niz slučajeva pacijenata sa PRS-om i linearnom sklerodermijom lica sa različitom prevalencijom, slično kao u slučaju naše pacijentkinje.…”

Section: Diskusijaunclassified

Parry Romberg syndrome with en coup de sabre: A report of a rare case

Faizal¹,

Babu²,

Shetty³

et al. 2021

Acta stomatologica Naissi

View full text Add to dashboard Cite

The basis of the problem: Parry Romberg syndrome (PRS) is a rare developmental disorder manifesting as a slow and progressive atrophy of the face which is often unilateral, hence also termed as rpogressivehemifacial atrophy. The extent of the atrophy may vary, involving the superficial skin extending upto the underlying bone. The clinical features of PRS include craniofacial, neurologic, ophthalmic and dermatological manifestations, which result in various functional and psychological problems. PRS and linear scleroderma belong to the same disease spectrum. There can be demarcating line between the normal and abnormal skin seen in PRS, termed as "en coup de sabre". PRS is most commonly seen in females and involves the left side of the face. Method: We hereby report a case of PRS in a young girl presenting with en coup de sabre appearance on the right side of face. Results: Microsurgical facial reconstruction of the affected side is known to be the gold standard method for correction of the facial symmetry. In our case, the patient is currently kept under long term follow up and cosmetic surgical treatment will be planned once the atrophy attains stability. Conclusion: The association of PRS with linear scleroderma may present difficulty in its diagnosis. Hence, careful recording of the history and clinical examination with appropriate investigations can aid in establishing the correct diagnosis.

show abstract

Parry–Romberg syndrome in an adolescent: a case report on progressive hemifacial atrophy

Cited by 6 publications

References 9 publications

Parry Romberg Syndrome: When the Diagnosis of a Rare Disease Is Made in the Primary Care Setting

Parry Romberg Syndrome: When the Diagnosis of a Rare Disease Is Made in the Primary Care Setting

Parry Romberg Syndrome: a case report

Parry Romberg syndrome with en coup de sabre: A report of a rare case

Contact Info

Product

Resources

About