Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

Wallach, Asya I.; Magro, Cynthia M.; Franks, Andrew G; Shapiro, Lee; Kister, Ilya

doi:10.1007/s11910-019-0929-8

Cited by 6 publications

(6 citation statements)

References 95 publications

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“…non-malignancy-associated SS as well as malignancy associated SS [6][7][8]. SS has also been reported to have no racial predilection, although neuro-Sweet disease, a variant of SS presenting with neurological symptoms, has been most commonly noted in Japanese patients [9].…”

Section: Key Pointsmentioning

confidence: 99%

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New Practical Aspects of Sweet Syndrome

et al. 2022

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Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous papules/plaques/pustules/nodules commonly appearing on the upper limbs, trunk, and head and neck; histologically, SS is characterized by dense neutrophilic infiltrate in the dermis. SS is accompanied by fever; an elevation of inflammatory markers (e.g., erythrocyte sedimentation rate, C reactive protein) in serum may also be observed. Although most cases of SS are idiopathic, SS also occurs in the setting of malignancy or following administration of an associated drug. SS has also been reported in association with pregnancy and a burgeoning list of infectious (most commonly upper respiratory tract infections) and inflammatory diseases; likewise, the litany of possible iatrogenic triggers has also grown. Over the past several years, a wider spectrum of SS presentation has been realized, with several reports highlighting novel clinical and histological variants. Corticosteroids continue to be efficacious first-line therapy for the majority of patients with SS, although novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory SS. New mechanisms of SS induction have also been recognized, although the precise etiology of SS still remains elusive. Here, we catalogue the various clinical and histological presentations of SS, summarize recently reported disease associations and iatrogenic triggers, and review treatment options. We also attempt to frame the findings of this review in the context of established and emerging paradigms of SS pathogenesis.

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Section: Key Pointsmentioning

confidence: 99%

“…The authors also reported certain cases of neuro-Sweet disease to present with neurovascular accidents, including strokes and ophthalmic vein thrombosis. Imaging and cerebrospinal fluid analyses are highly variable across cases, and therefore of little diagnostic value [9].…”

Section: Neuro-sweet Diseasementioning

confidence: 99%

New Practical Aspects of Sweet Syndrome

et al. 2022

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“…Both SIADH and NDS improved after second-line treatment, which led to the hypothesis that cytokines such as interleukin IL-6 may play a part in NSD and SIADH [ 23 , 24 ]. Additional neurological symptoms for neuro-Sweet have been reported: aphasia, ataxia, hemisensory loss, focal seizures, hemiparesis or movement disorder [ 25 ].…”

Section: Resultsmentioning

confidence: 99%

Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach

Ferea

Mihai

Bălan

et al. 2023

Life

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Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients.

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“…5 SS can present as an idiopathic disease or in association with drugs, such as colony stimulating factors, oral tretinoin, chemotherapies and antimicrobials; infections, mainly involving the respiratory and gastrointestinal tract, malignancy, inflammatory conditions, and pregnancy. 6 It can affect multiple organs besides the skin including liver, heart, lung, mouth, bones, central nervous system although most commonly affect the eyes in 17-72% of cases. 1 Neurologic involvement is uncommon and has been mainly reported in Asian population.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, the few pathological reports of central nervous tissue in NSS demonstrated neutrophilic infiltrate of blood vessels with vasculitis probably as a manifestation of an active process underlying the disease. 6,8 The diagnosis of NSS can be difficult and requires a high degree of suspicion. In 2005, Hisanaga developed diagnostic criteria based on a cohort of Japanese patients.…”

Section: Discussionmentioning

confidence: 99%

Neuro-Sweet Syndrome: A Diagnostic Conundrum

Acurio,

Chuquilin

2023

The Neurohospitalist

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Sweet Syndrome presents as acute fever, leucocytosis and characteristic skin plaques. It can involve many organ systems but rarely affects the nervous system. We report the case of a 51-year-old female that presented with fever, rash, headache and encephalopathy. Brain magnetic resonance imaging showed extensive T2 hyperintensities involving cerebral hemispheres, cerebellum, and brainstem. A skin biopsy revealed dermal infiltration by neutrophils consistent with Sweet Syndrome. She started steroid treatment with a good clinical response. Further questioning revealed that she had a similar episode 10 years prior that had been diagnosed as acute disseminated encephalomyelitis. Neuro-Sweet Syndrome can present with a great array of symptoms and relapses over long periods of time making the diagnosis difficult without a high degree of suspicion. Clinicians should consider this syndrome in the setting of acute encephalitis with white matter lesions that are highly responsive to steroids particularly in the presence of previous similar symptoms.

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Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

Cited by 6 publications

References 95 publications

New Practical Aspects of Sweet Syndrome

New Practical Aspects of Sweet Syndrome

Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach

Neuro-Sweet Syndrome: A Diagnostic Conundrum

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