CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities

Doolittle, Derrick A.; Lehman, Vance T.; Schwartz, Kara M.; Wong‐Kisiel, Lily C.; Lehman, Julia S.; Tollefson, Megha M.

doi:10.1007/s00234-014-1448-6

Cited by 58 publications

(63 citation statements)

References 48 publications

(67 reference statements)

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“…In fact the association between Parry-Romberg and the S. Sjogrenreopens the discussion about autoimmunity because, in our case, the patient is suffering simultaneously from a localized connectivitis, as Parry-Romberg, and a systemic connectivitis, as Sjogren Syndrome [7]. Laboratory examinations showed negative antibody.…”

Section: Methodsmentioning

confidence: 57%

“…Subsequently, it appears a scar which extends along the median part of the face, called "coup de sabre" [7].…”

Section: Introductionmentioning

confidence: 99%

“…[3] Also, Parry-Romberg Syndrome is usually not observed in other autoimmune disease, including Sjogren Syndrome, which is a chronic inflammatory autoimmune disease characterized by destruction of exocrine glands, in particular lacrimal and salivary glands [3,6,8].…”

Section: Introductionmentioning

confidence: 99%

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The Late Demonstration of the Parry-Romberg Syndrome

Persechino¹

2016

CRDOA

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We report the case of a 46 years-old woman, who developed a linear lesion sclero-atrophic from the medial frontal region and which expanded in cranio-caudal sense, with subsequent skin and dermic atrophy.A biopsy was performed and confirmed a dermal-epidermal sclerosis. These data, associated with the clinical characteristics, let us to diagnose a Parry-Romberg Syndrome.Parry Romberg syndrome is a scleroatrophic syndrome, linear of the face which presents a localized partial hemiatrophy without systemic involvement.In addition, the patient reported diffuse pain in the joints, previous autoimmune thyroiditis and reduced salivation and lacrimation bilaterally with the positive Schirmer test. The data confirmed the hypothesis of a possible S. Sjogren associated with Parry-Romberg. This medical case is suggestive because the Parry-Romberg is a rare syndrome in adults, and usually not observed in other autoimmune disease, as S. Sjogren.

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Section: Methodsmentioning

confidence: 57%

“…Subsequently, it appears a scar which extends along the median part of the face, called "coup de sabre" [7].…”

Section: Introductionmentioning

confidence: 99%

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The Late Demonstration of the Parry-Romberg Syndrome

Persechino¹

2016

CRDOA

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“…The above features of NH reminded us of Parry‐Romberg syndrome (PRS), which is characterized by progressive hemiatrophy of skin and soft tissue of the face innervated by the trigeminal nerve . PRS has been regarded as a neurocutaneous syndrome in consideration of the cutaneous lesions and neurologic symptoms, which are the most common systemic symptoms associated with PRS . Headache is one of the most frequently reported neurological symptoms .…”

Section: Discussionmentioning

confidence: 99%

Nummular Headache: 2 Cases With Good Beta Blocker Responses and a Narrative Review

Jiang

Liu

et al. 2019

Headache

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Objective To report 2 new nummular headache (NH) cases and review the clinical features and management of NH. Background NH is a coin‐shaped headache with a fixed shape and size. Since the first case series was reported by Pareja, approximately 300 cases have been reported. However, the latest treatment status and pathogenesis of NH remain unclear. Methods Two new NH cases with improved symptomatic responses to metoprolol were reported. We also conducted a narrative review of cases published in English aiming to provide the newest information about the clinical features and management of NH. Results Two NH patients with skin depressions within the painful area experienced significantly improvement with metoprolol. The literature review showed that NH was mainly moderate‐to‐severe in 77.8% (186/239) of the patients. Sensory dysfunction and focal trophic changes were reported in 47.8% (149/312) and 3.8% (12/312) of the patients, respectively. Gabapentin was the most commonly used and effective prophylactic, with a responder rate of 67.9% (55/81). Botulinum toxin type A was used for refractory NH, with an effective rate of 81.8% (9/11). Ten patients from the previous studies were prescribed β‐adrenergic blockers without any relief, and none of them reported focal tropic changes. Conclusions NH can be a disabling headache in more than half of identified patients and requires medical intervention. As with the previous treatment recommendations, gabapentin was the most commonly used prophylactic with a good response. Our cases suggested that β‐adrenergic blockers could be a therapeutic option for NH patients with focal trophic changes within the areas.

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“…Odontostomatologic complications should be managed accordingly in cooperation with an oral maxillofacial surgeon and/or dentist [33–35]. Lastly, neurological manifestations consisting of seizures and headaches [145] may occur and referral to a neurologist could be considered to rule out central nervous system involvement [32, 146–150]. …”

Section: Referral and Flow Chartmentioning

confidence: 99%

Morphea and Eosinophilic Fasciitis: An Update

et al. 2017

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Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud’s phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

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CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities

Abstract: In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.

Cited by 58 publications

References 48 publications

The Late Demonstration of the Parry-Romberg Syndrome

The Late Demonstration of the Parry-Romberg Syndrome

Nummular Headache: 2 Cases With Good Beta Blocker Responses and a Narrative Review

Morphea and Eosinophilic Fasciitis: An Update

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