Giant cellulitis-like Sweet syndrome as an initial clinical presentation of acute myeloblastic leukemia with t(6;9)(p23;q34): DEK-CAN and internal duplications of FMS-like tyrosine kinase 3

Okuyama, Shuhei; Nito, Toshiya; Yanagawa, Naoki; Tajima, Katsushi

doi:10.1007/s00277-019-03613-1

Cited by 4 publications

(4 citation statements)

References 5 publications

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“…However, cultures of cellulitislike SS are negative for pathogenic organisms, and antibiotic treatment is unable to induce lesion remission. Three recent cases of cellulitis-like SS have been reported, and the authors of all cases concede that the diagnosis of cellulitislike SS is challenging [39][40][41]. Thus, clinicians should maintain a high index of suspicion for SS when a cellulitis-like clinical picture presents with negative bacterial cultures and displays no response to antibiotic therapy.…”

Section: Cellulitis-like Sweet Syndromementioning

confidence: 99%

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: Cellulitis-like Sweet Syndromementioning

confidence: 99%

New Practical Aspects of Sweet Syndrome

et al. 2022

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“…Cellulitis-like SS is a rare SS variant defined by tender, erythematous, edematous lesions, hardly distinguishable from bacterial cellulitis. Differential diagnosis is ensured by negative cultures for pathogenic organisms and a lack of response to antibiotic treatment [ 18 , 47 , 48 , 49 ].…”

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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“…The lesions of GC-SS are characterized by recurrent, abrupt appearance of large, well-defined infiltrated plaques, with bullous appearance and involving multiple skin areas[ 1 - 5 ]. However, the characteristics of our patient’s lesion differed from those previously described; namely, it was solitary and progressively expanding, making it much more difficult to distinguish from cellulitis.…”

Section: Discussionmentioning

confidence: 99%

“…According to available case reports, GC-SS tends to occur in patients with obesity, autoimmune diseases, and malignancies, including hematologic malignancies, such as myelodysplastic syndrome (MDS), multiple myeloma, and acute myeloid leukemia. The relationship between GC-SS and MDS remains to be elucidated[ 2 - 5 ].…”

Section: Introductionmentioning

confidence: 99%

Histiocytoid giant cellulitis-like Sweet syndrome at the site of sternal aspiration: A case report and review of literature

Zhao¹,

Ni²,

Sun³

2022

WJCC

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BACKGROUND Giant cellulitis-like Sweet syndrome (SS) is a rare subtype of SS, and reports of the combined histiocytoid type of pathology are scarce. Here, we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis. By sharing this case and a discussion of the related literature in detail, we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like (HGC)-SS and the pathogenesis of SS. CASE SUMMARY A 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swelling with pain for 1 d. He was diagnosed with myelodysplastic syndrome (MDS) and angioneurotic edema of the tongue and floor of the mouth. However, 7 d after examination by sternal aspiration, a violaceous, tender, and swollen nodule developed at the site, with poorly demarcated erythema of the surrounding skin. Considering his profile of risk factors, the diagnosis of cellulitis was made and he was administered broad-spectrum antibiotics. When the lesion continued to worsen and he developed chills and fever, pathogenic and dermatopathological examination led to the diagnosis of HGC-SS. Treatment with prednisone led to the fever being relieved within 24 h and the skin lesion being resolved within 1 wk. The patient refused intensive treatment and was instead given thalidomide, erythropoietin, stanozolol, and supportive care. The prednisone was gradually tapered, with no signs of recurrence, but he died 2 mo later of severe pneumonia. CONCLUSION HGC-SS demonstrates unique manifestation. SS and leukemia cutis share cytological origin. Myelofibrosis and SS are adverse prognostic factors for MDS.

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Giant cellulitis-like Sweet syndrome as an initial clinical presentation of acute myeloblastic leukemia with t(6;9)(p23;q34): DEK-CAN and internal duplications of FMS-like tyrosine kinase 3

Cited by 4 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

Histiocytoid giant cellulitis-like Sweet syndrome at the site of sternal aspiration: A case report and review of literature

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