Early Features of Progressive Hemifacial Atrophy—Clinical and Imaging Findings

Jones, Krystal M.; Tan, Jennifer K.; Alomari, Ahmad I.; Son, Mary Beth; Vleugels, Ruth Ann; Mulliken, John B.; Liang, Marilyn G.

doi:10.1001/jamadermatol.2020.2593

Cited by 3 publications

(2 citation statements)

References 6 publications

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“…Clinically, the initial signs are thinning of the skin and subcutaneous tissues, usually preceded by alopecia areata, or vitiligo as seen in our patient [ 1 , 6 ]. The syndrome usually progresses slowly over a variable course from two to 20 years, reaching a “burned-out phase” and stabilizes [ 1 , 3 , 9 , 11 , 15 ]. Our patient is being followed up.…”

Section: Discussionmentioning

confidence: 99%

Parry Romberg Syndrome in a Young Ghanaian: A Case Report and a Literature Review

Dzefi-Tettey¹,

Edzie²,

Seadey³

et al. 2022

Cureus

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Parry Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a very rare self-limiting disease, which affects the skin and subcutaneous tissues, underlying musculature, cartilage, and bony structures of one half of the face with a resultant hemiatrophy and alopecia areata. It presents in children and young adults, with a slow progression of the atrophy for several years, and then becomes stable. Magnetic resonance imaging (MRI) or computed tomography (CT) scan of the cranium demonstrates the radiological feature of hemiatrophy very clearly. We report a case of PRS in a nine-year-old girl with characteristic features which was diagnosed based on medical history, clinical signs, and radiological findings on cranial CT scan and MRI.

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Section: Discussionmentioning

confidence: 99%

Parry Romberg Syndrome in a Young Ghanaian: A Case Report and a Literature Review

Dzefi-Tettey¹,

Edzie²,

Seadey³

et al. 2022

Cureus

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“…8 It can present with varying degrees of atrophy involving skin, subcutaneous tissue, and bone, and ocular and central nervous system findings may also be present. [8][9][10][11][12] Following a period of disease activity, the atrophy may stabilize, but recurrent flares are known to occur. There are a number of oral health concerns in Parry-Romberg Syndrome, reported primarily in small series documenting findings that involve soft tissue (ie tongue atrophy, gingival exposure, lip incompetence), the jaws (ie temporomandibular joint dysfunction, malocclusion), and teeth (ie agenesis, delayed eruption, and root atrophy).…”

Section: Introductionmentioning

confidence: 99%

Dental Anomalies in Parry-Romberg Syndrome: A 40-Year Retrospective Review

Aram

Cappitelli

Dedeoğlu

et al. 2022

The Cleft Palate Craniofacial Journal

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Objective This study investigates crown and root anomalies in patients with Parry-Romberg Syndrome. Design This is a retrospective review of patients with Parry-Romberg Syndrome who were evaluated at a tertiary care center from 1980-2020. Setting Patients seen in the dental unit from 1980-2020. Patients, Participants Seventeen patients with documented Parry-Romberg Syndrome were referred for dental evaluation. Main Outcome Measures All dental anomalies were documented. Root anomalies were assessed using panoramic radiographs and cone beam CT (CBCT) scans to evaluate buccal-lingual, mesio-distal, and axial measurements of hypoplastic teeth, which were compared to those of contralateral teeth. Results Findings included agenesis (29%, n = 5), hypoplastic teeth (29%, n = 5), delayed canine eruption (24%, n = 4), and mulberry molars (12%, n = 2). Of the five patients with tooth hypoplasia, four had CBCT records and the fifth had panoramic radiographs available for assessment. Axial length was always shorter in hypoplastic teeth relative to contralateral teeth, with differences ranging from 1.2-9.2 mm. Differences in crown size of hypoplastic versus contralateral teeth were unpredictable but always present. Conclusions Patients with Parry-Romberg Syndrome can have hypoplastic roots with atypical crown morphology. A patient’s specific dental anomaly will influence planning and treatment.

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Usefulness of cutaneous ultrasound in the management of Parry Romberg syndrome

Quintana‐Castanedo

Sánchez-Orta

Nuño-González

et al. 2021

Skin Research and Technology

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Parry Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a rare craniofacial disorder characterized by unilateral facial atrophy affecting the skin, subcutis, muscle, and bones. 1 PRS is considered a severe variant of linear morphea, sharing some features with en coup de sabre scleroderma.Diagnosis is often delayed 1 and clinical monitoring is challenging.Visible changes are usually subtle, and decisions are often based on subjective impressions from the patient or physician.

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Early Features of Progressive Hemifacial Atrophy—Clinical and Imaging Findings

Abstract: Author Contributions: Drs Shields and Rosenbach had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: Shields, Gelfand, Rosenbach.

Cited by 3 publications

References 6 publications

Parry Romberg Syndrome in a Young Ghanaian: A Case Report and a Literature Review

Parry Romberg Syndrome in a Young Ghanaian: A Case Report and a Literature Review

Dental Anomalies in Parry-Romberg Syndrome: A 40-Year Retrospective Review

Usefulness of cutaneous ultrasound in the management of Parry Romberg syndrome

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