Síndrome de Sweet: estudo de 73 casos, com ênfase nos achados histopatológicos

Abstract

BACKGROUND: Sweet's syndrome refers to a set of cutaneous, systemic and histopathological alterations that occur in response to different stimuli, in a similar way to that occurring in erythema nodosum, erythema multiforme and leukocytoclastic vasculitis. The syndrome has been described in association with conditions such as infections, pregnancy, the use of certain medications and malignancy. OBJECTIVES: To evaluate the clinical and histopathological alterations occurring in this syndrome and to assess the association between these alterations and other conditions. METHODS: A retrospective study of 73 cases was conducted, evaluating data on the microscopic examination of skin lesions, as well as clinical and laboratory data. RESULTS: The majority of the patients were female (83.0%), white (49.2%) and between 30 and 60 years of age (73.8%). The principal alterations found were: erythymatous plaques (76.9%), papules (43.0%), pseudo-vesiculation (PV) (38.4%) and target lesions (18.5%). With respect to the associated conditions, upper respiratory tract infections (15.4%) and the use of medication (10.8%) were the most common. Other associations, albeit represented by only one case each, were: Hodgkin's lymphoma, pregnancy, ulcerative colitis, polycythemia vera and lupus erythematosus in a patient with acquired immune deficiency syndrome (AIDS). The principal microscopic findings were: neutrophils with leukocytoclasia (98.6%), collagen degeneration (87.7%), edema (74.0%) and PV (38.4%). The presence of eosinophils (41.1%) ranged from rare to abundant and was usually unrelated to the use of medication. Inflammatory infiltrate reached the deep epidermal layer in 47.9% of cases and panniculitis was found in 80.0% of cases in which the hypodermis was affected (10 cases). CONCLUSIONS: In general, these findings are in agreement with results published in the literature, emphasizing the frequent finding of eosinophils unrelated to drug use, panniculitis and the rare association with Hodgkin's lymphoma. This is the fifth report of an association between Sweet's syndrome and Hodgkin's disease. Keywords: Erythema Multiforme; Hodgkin's Disease; Panniculitis; Sweet's Syndrome.Resumo: FUNDAMENTOS: A síndrome de Sweet corresponde a um conjunto de alterações cutâneas, sistêmicas e histopatológicas como resposta a diversos estímulos, semelhantes ao eritema nodoso, ao eritema multiforme e à vasculite leucocitoclásica. São descritas condições associadas como infecção, gravidez, uso de drogas e malignidades. OBJETIVOS: Avaliar as alterações clínicas e histopatológicas da síndrome, relacionando-a a outras condições. MÉTODO: Estudo retrospectivo de 73 casos com avaliação microscópica de lesões cutâneas, dados clínicos e laboratoriais. RESULTADOS: Houve predomínio de mulheres (83,0%), brancas (49,2%), entre a quarta e a sexta décadas de vida (73,8%). Placas eritematosas (76,9%), pápulas (43,0%), pseudovesiculação (PV) (38,4%) e lesões em alvo (18,5%) foram as principais alterações. Entre as condições associadas, infecções de vi...

Insights Into the Characteristics of Sweet Syndrome in Patients With and Without Hematologic Malignancy

“…In this study, we observed that Sweet syndrome was more frequent in the women, and the mean age at diagnosis was 53 years, consistent with the data described in the literature (10)(11)(12)(13). Our results failed to show significant differences in age and sex in the patients of Sweet syndrome with and without hematologic malignancy.…”

“…In our cohort, fever was notably more common than other clinical symptoms (24/37, 65%), with a higher incidence than that in some studies (11)(12)(13), emphasizing that fever exists as an important diagnostic criterion of Sweet syndrome (3,4). Our study obtained a positive association with arthralgia in only 5% of the patients, none of whom had Sweet syndrome with hematologic malignancy.…”

Sweet Syndrome in an Elderly Man With Well-Controlled Human Immunodeficiency Virus

“…We performed a literature search using PubMed with search terms "HIV AND Sweet" or "HIV AND Acute febrile neutrophilic dermatosis," and identified 17 other published cases from 1994 to 2020 of Sweet syndrome in patients with HIV infection ( Table 1). One of these cases is mentioned in the context of a larger study on histopathological findings, but no further supporting details were available for tabulation [3]. A review of the published cases highlights important unique proposed etiologies for Sweet syndrome in those living with HIV: immune reconstitution inflammatory syndrome (IRIS) in 25% of cases [6,13,17], AIDS-defining conditions (i.e., Kaposi sarcoma and pneumocystis pneumonia) in two cases [5,6], and abacavir therapy in one case [8].…”

Sight-Threatening Ocular Manifestations of Acute Febrile Neutrophilic Dermatosis (Sweet's Syndrome)

“…The head, neck and upper extremities are favored in an often asymmetric distribution, but lesions can occur anywhere on the body. Histologically, extensive papillary dermal edema with a diffuse nodular and perivascular neutrophilic infiltrate is evident [7]. A peripheral leukocytosis with neutrophilia and elevated ESR and CRP can help narrow the diagnosis.…”