Sweet syndrome with pulmonary involvement in a patient with myelodysplastic syndrome

Mizes, Alicia; Khosravi, Hamed; Bordelon, Jenna R.; Kazlouskaya, Viktoryia; Karunamurthy, Arivarasan; Ho, Jonhan; James, Alaina J.; Patton, Timothy

doi:10.5070/d3263048029

Cited by 3 publications

(4 citation statements)

References 7 publications

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“…In addition to the common manifestations, patients may present with pulmonary involvement, a rare complication with only 45 cases reported in the literature and only 1 case published in a dermatologic issue [ 15 ].…”

Section: Resultsmentioning

confidence: 99%

“…Herein, Nofal A. et al reviewed the clinical, laboratory, and histopathological aspects of the diagnostic criteria for SS and evaluated their validity in the reported literature and in 40 new patients, subsequently proposing certain modifications of the diagnostic criteria ( Table 3 ) [ 35 ]. Additional minor criteria for diagnosis include fever (40–80% of patients), increased erythrocyte sedimentation rate, high values for C-reactive protein, neutrophilic leukocytosis, responsiveness to systemic corticosteroids therapy, and the presence of a chronic inflammatory disorder, such as infection, pregnancy, malignancy, drug exposure, or myeloproliferative disorders [ 15 ]. The diagnosis is based on both major criteria and at least two minor criteria [ 9 ].…”

Section: Resultsmentioning

confidence: 99%

“…Other cells’ presence has been observed, such as eosinophils, on skin biopsies in classical or drug-induced variants of the syndrome [ 13 ] or lymphocytes or histiocytes in rare cases [ 50 , 56 ]. The presence of a perivascular neutrophilic infiltrate may lead to leukocytoclastic vasculitis [ 15 , 57 ], a characteristic aspect of postcapillary venules. This is defined by neutrophils with nuclear dust, extravasation of erythrocytes, necrosis, and granuloma formation, with fibrin deposits in vessel walls [ 58 ].…”

Section: Resultsmentioning

confidence: 99%

“…At admission, patient 4 [ 15 ] presented severe anemia and low platelets on CBC, sustaining a very high ESR, all in the context of extensive pulmonary ground glass opacities on CT scan and significant skin lesion development. Interdisciplinary collaboration helped completely evaluate this patient with pulmonary involvement on account of MDS.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

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

Ferea

Mihai

Bălan

et al. 2023

Life

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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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