Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Abdelnour, Jimmy Girgis William; Abdelnour, Youeil Girgis William; Kerollos, Rose-Mery Amin Boushra; Mahmoud, Ziryab Imad Taha

doi:10.1186/s13256-019-2063-2

Cited by 9 publications

(15 citation statements)

References 19 publications

(20 reference statements)

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“…Hair defects such as focal baldness was also seen in our case. Common maxillofacial manifestations include deviation of nose and mouth to the affected side as observed in our case, but other maxillofacial features like atrophy of the tongue, underdeveloped jaws, hard and soft palate, delayed tooth eruption and teeth with short roots were not present in our case 10 . Retinal vasculitis and loss of periorbital fat resulting in enophthalmos are the common ophthalmological manifestations.…”

Section: Discussionmentioning

confidence: 53%

“…Microsurgical facial reconstruction of the affected side is known to be the gold Obrazovanje pacijenata, motivaciju i hiruršku korekciju atrofije treba uzeti u obzir, kada je reč o uspešnom ishodu lečenja. Poznato je to da je mikrohirurška rekonstrukcija lica zahvaćene strane zlatni standard među metodama za korekciju asimetrije lica 10 . Kozmetičke hirurške opcije uključuju mikrovaskulature slobodne režnjeve, aloplastične graft materijale za transplantaciju, autolognu transplantaciju masti itd.…”

Section: Discussionunclassified

“…Defekti kose, poput fokalne alopecije, takođe su primećeni u slučaju naše pacijentkinje. Uobičajene maksilofacijalne manifestacije uključuju promene u predelu nosa i usana na zahvaćenoj strani, što je primećeno i u slučaju naše pacijentkinje, ali druge maksilofacijalne karakteristike, kao što su atrofija jezika, nerazvijene obe vilice, tvrdo i meko nepce, odloženo nicanje zuba i to sa kratkim korenom nisu detektovani u slučaju pacijentkinje predstavljene u ovom radu 10 . Mogu se javiti i retinalni vaskulitis i gubitak periorbitalnog masnog tkiva, koji rezultira enoftalmosom, a česte su i oftalmološke manifestacije.…”

Section: Diskusijaunclassified

“…Nema standard method for correction of the facial symmetry 10 . Cosmetic Surgical options include Microvascular free flaps, Alloplastic graft materials, autologous fat transplantation, etc 11 .…”

Section: Sukobi Interesamentioning

confidence: 99%

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

Faizal¹,

Babu²,

Shetty³

et al. 2021

Acta stomatologica Naissi

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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.

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Section: Discussionmentioning

confidence: 53%

Section: Discussionunclassified

Section: Diskusijaunclassified

Section: Sukobi Interesamentioning

confidence: 99%

See 2 more Smart Citations

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

Faizal¹,

Babu²,

Shetty³

et al. 2021

Acta stomatologica Naissi

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“… 1 To the best of our knowledge, less than 100 cases of patients with AO linear scleroderma ECDS have been reported. 2 , 4 , 5 – 17 …”

Section: Discussionmentioning

confidence: 99%

Adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda: A case report and literature review

Mititelu

Ridha

Ouchene

et al. 2021

SAGE Open Medical Case Reports

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The en coup de sabre variant of linear scleroderma typically occurs in children. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. The patient was treated with oral steroids and oral methotrexate improving her cutaneous disease. This case highlights the importance of a thorough cutaneous examination as this adult patient developed an entity traditionally believed to occur in childhood.

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A case report of Parry–Romberg syndrome

Babaei,

Rahnama,

Shurvarzi

et al. 2024

Clinical Case Reports

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Key Clinical MessageParry–Romberg syndrome is characterized by progressive dystrophy in one half of the face, which usually begins in childhood. Correct and timely diagnosis of this disease, as well as a multidisciplinary approach and timely surgical treatment to minimize the psychological effects and improve the patient's appearance are of particular importance.AbstractParry–Romberg syndrome is characterized by progressive dystrophy or loss of subcutaneous tissue in one half of the face, which usually begins in childhood and continues with skin changes, and can also be associated with linear scleroderma. Although this disease has been known for more than 150 years, its exact cause and pathogenesis are not well understood. The clinical feature of Parry–Romberg syndrome that makes it possible to diagnose is unilateral idiopathic facial atrophy. The reported case is a 14‐year‐old boy who suffered from hemifacial atrophy of the frontal area since he was 7 years old was referred to a plastic and cosmetic surgery specialist and underwent surgery without systemic symptoms and in the inactive phase of the disease. Correct and timely diagnosis of this disease, as well as a multidisciplinary approach and timely and appropriate surgical treatment to minimize the psychological effects and improve the patient's appearance are of particular importance.

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Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Cited by 9 publications

References 19 publications

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

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

Adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda: A case report and literature review

A case report of Parry–Romberg syndrome

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