Normolipemic xanthomatized Sweet’s syndrome: A variant of Sweet’s syndrome with myelodysplastic syndrome

Kamimura, Anna; Yanagisawa, Hiroto; Tsunemi, Yuichiro; Kusano, Takeru; Arai, Eiichi; Tsuchida, Takaaki; Nakamura, Koichiro

doi:10.1111/1346-8138.15781

Cited by 6 publications

(5 citation statements)

References 5 publications

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“…Recently, a novel subtype of SS, normolipemic xanthomized Sweet syndrome, was reported by Kamimura et al Microscopic examination of this unusual case revealed CD163-positive foam cells to accompany dense neutrophilic infiltrate extending into the deep dermis [69]. Further reports may aid in further characterization of this variant.…”

Section: Other Histologic Patternsmentioning

confidence: 73%

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: Other Histologic Patternsmentioning

confidence: 73%

New Practical Aspects of Sweet Syndrome

et al. 2022

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“…Another unusual case reported was that of a patient with Sweet syndrome characterized by normolipemic xanthoma-infiltrated neutrophilic dermatosis developed with myelodysplastic syndrome (MDS) with single-lineage dysplasia [ 65 ].…”

Section: Resultsmentioning

confidence: 99%

“…A new and rare variant of SS, normolipemic xanthomized Sweet syndrome (A. Kamimura et al [ 65 ]), clearly discloses that SS classification is not near to completion, while SS pathogenesis has a few starting theories. This unique case describes a prolonged evolution with adverse outcomes, overlooking the MDS that is affiliated with a good prognosis.…”

Section: Discussionmentioning

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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“…There have been other clinicopathogical variants reported in the literature in the setting of MDS/MPN mentioned above [17]. Among all of these variants, it is worth highlighting the subcutaneous Sweet syndrome (S-SS) or neutrophilic lobular panniculitis (Figure 1D-F) [18,19].…”

Section: Other Morphological Variants Of the Sweet Syndromementioning

confidence: 95%

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

Prieto-Torres,

Requena,

Rodríguez-Pinilla

2023

Cancers

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Myeloid neoplasms and acute leukemias include different entities that have been recently re-classified taking into account molecular and clinicopathological features. The myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) category comprises a heterogeneous group of hybrid neoplastic myeloid diseases characterized by the co-occurrence of clinical and pathological features of both myelodysplastic and myeloproliferative neoplasms. The most frequent entity in this category is chronic myelomonocytic leukemia (CMML) which is, after acute myeloid leukemia (AML), the main myeloid disorder prone to develop cutaneous manifestations. Skin lesions associated with myelodysplastic and myeloproliferative neoplasms include a broad clinical, histopathological and molecular spectrum of lesions, poorly understood and without a clear-cut classification in the current medical literature. The aim of this review is to describe and classify the main clinical, histopathological and molecular patterns of cutaneous lesions in the setting of MDS/MPN in order to improve the diagnostic skills of the dermatologists, hematologists and pathologists who deal with these patients.

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Normolipemic xanthomatized Sweet’s syndrome: A variant of Sweet’s syndrome with myelodysplastic syndrome

Cited by 6 publications

References 5 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

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

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