Systemic sarcoidosis presenting as Harlequin and Horner syndrome

Wong, Janice; Doyle, Tracy J.; Paulson, Vera; Milligan, Tracey; Prasad, Sashank

doi:10.1212/cpj.0000000000000192

Cited by 5 publications

(6 citation statements)

References 5 publications

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“…To the best of our knowledge, this is the second described case of sarcoidosis-induced Harlequin syndrome. Wong et al [ 17 ] presented the case of a 61-year-old woman with systemic sarcoidosis that manifested as Harlequin and Horner syndromes. In that case, the clinical presentation of sympathetic dysfunction overshadowed the most common respiratory and nonspecific symptoms of sarcoidosis such as cough, dyspnea, fatigue, fever, and weight loss.…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of sarcoidosis-associated Harlequin syndrome may be explained by several mechanisms. In Wong et al’s case, they supposed it was caused by compression of the sympathetic chain due to parenchymal lung disease or mediastinal lymphadenopathy [ 17 ]. In our case, the patient’s radiological studies also showed enlarged mediastinal lymph nodes.…”

Section: Discussionmentioning

confidence: 99%

“…Facial and optic neuropathies are seen most frequently, although any part of the nervous system can be affected [ 16 ]. Research shows that up to 30% of patients with sarcoidosis suffer from small-fiber neuropathy [ 15 ], whereas there is only anecdotal data about the incidence of ganglionopathy and Harlequin syndrome in this population [ 17 ].…”

Section: Introductionmentioning

confidence: 99%

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Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Navickaitė,

Ališauskienė,

Petrauskienė

et al. 2023

Medicina

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Background and Objectives: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. Case presentation: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. Conclusions: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Navickaitė,

Ališauskienė,

Petrauskienė

et al. 2023

Medicina

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“…Otras causas menos frecuentes son: las enfermedades tiroideas y la sarcoidosis. 18,19 Esporádicamente, el signo de arlequín es visto como la presentación inicial de enfermedades como atrofia de múltiples sistemas, neuropatía diabética y falla autonómica pura. 20 La fisiopatología del síndrome de arlequín de origen idiopático o primario cuenta con distintas hipótesis: algunos autores sugieren que el daño puede ser ocasionado por isquemia microvascular, por alteración autoinmune o posterior a infecciones.…”

Section: Discussionunclassified

Síndrome de Arlequín

Alvis-Castaño¹,

Arellano-Lozano²,

Vieyra-Medina³

2022

revecuatneurol

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Se trata de una mujer de 37 años, quien acudió por presentar eritema hemifacial derecho y diaforesis posterior a la realización de ejercicio y eventos estresantes de un año de evolución. Mediante la exploración física y los estudios paraclínicos se descartaron causas estructurales. Por lo tanto, se otorgó el diagnóstico de síndrome de arlequín. Se explicaron las medidas terapéuticas a la paciente y se le dio seguimiento.

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“…4 Among the secondary causes, it is necessary to rule out space-occupying lesions, especially neuroblastoma in children, as well as ischemic causes. 5 Complications of surgery and interventions are the main iatrogenic causes, in which erythema and hemifacial hyperhidrosis are temporary, disappearing in hours or days. 6 Although less common, other dysautonomic disorders can be associated with HS.…”

Section: Diagnosis: Congenital Harlequin and Horner Syndromes Discussionmentioning

confidence: 99%

Hemifacial erythema and sweating after exercise in a child

Sánchez‐Martínez

Moneva-Leniz

Gegúndez-Hernández

et al. 2020

Pediatric Dermatology

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A 9-year-old boy presented with a one-year history of several episodes of erythema and increased sweating on the right side of his face that appeared immediately after intense physical exercise. The skin findings were not present at the time of consultation. A photograph of one of the episodes was provided by the parent showing right hemifacial erythema (Figure 1). Physical examination, however, revealed contralateral anisocoria, miosis, mild hypochromia of the iris, ptosis measuring approximately 2 mm, as well as slight enophthalmos of the left eye ( Figure 2). These ocular features were permanent and had been present since birth. What is the diagnosis?

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Systemic sarcoidosis presenting as Harlequin and Horner syndrome

Cited by 5 publications

References 5 publications

Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Síndrome de Arlequín

Hemifacial erythema and sweating after exercise in a child

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