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A leprosy reaction resembling Sweet syndrome was first described in 1987. This cutaneous manifestation can be classified as the type 2 reaction which arises from antigen-antibody interaction. It can occur in patients with diagnosed or undiagnosed leprosy, and men with borderline leprosy tend to exhibit this type of reaction. Triggering factors may include WHO multibacillary treatment or prescription antibiotics. Several reports of this clinical phenomenon have been published, making physicians consider it as part of this spectrum of the disease. Treatment regime can include systemic steroids and thalidomide.

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Abstract: Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of Sweet's syndrome. Although the etiology is unknown, there are reports of association with infections, neoplasias, autoimmune diseases and medications. Histopathology shows a dense neutrophilic inflammatory infiltrate in the dermis. Treatment is based on the administration of systemic corticosteroids; however, a combination of medications is useful, given the frequency of relapses. The authors report a classic and clinically exuberant case of neutrophilic dermatosis of the dorsal hands, with excellent response to oral dapsone treatment, and offer a brief literature review.

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BACKGROUND: Histiocytoid Sweet syndrome is characterized by a predominant neutrophilic dermal infiltrate. Usual clinical differential diagnosis includes erythema multiforme, drug eruption, and erythema nodosum. Histiocytoid Sweet syndrome is considered an uncommon histopathological variant of the disease. METHODS: We evaluated clinical, histopathological, and immunohistochemical findings of a case categorized as idiopathic histiocytoid Sweet syndrome in which clinical-epidemiological data raised the possibilities of Sweet syndrome, leprosy, and drug reaction. RESULTS: Positive reaction to myeloperoxidase (MPO) in histiocytoid cells of the dermal infiltrate, response to oral corticosteroids, clinical and laboratory investigation, and absence of cutaneous lesions or clinical complaints within 1 year of follow-up are consistent with the diagnosis of idiopathic histiocytoid Sweet syndrome. CD68 (PG-M1) and CD15 positive cells were also present among dermal cells. CONCLUSIONS: Epidemiological data are relevant while considering a clinical differential diagnosis of Sweet syndrome that can be further expanded, from a histopathological point of view, when dealing with the histiocytoid variant since neutrophils, macrophages, and immature myelomonocytic cells with histiocytoid morphology are present. The significance of the MPO positive mononuclear dermal cells are not completely established.

Assuntos

RESUMO

Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of Sweet's syndrome. Although the etiology is unknown, there are reports of association with infections, neoplasias, autoimmune diseases and medications. Histopathology shows a dense neutrophilic inflammatory infiltrate in the dermis. Treatment is based on the administration of systemic corticosteroids; however, a combination of medications is useful, given the frequency of relapses. The authors report a classic and clinically exuberant case of neutrophilic dermatosis of the dorsal hands, with excellent response to oral dapsone treatment, and offer a brief literature review.

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RESUMO

Sweet (SS) syndrome is a neutrophilic dermatosis, characterized by a rapid onset of painful erythematous-purplish papules, nodules or plaques, accompanied by fever and associated with a dermal neutrophil infiltrate. CASE REPORT: Male, 69 years old, with a history of mild plaque psoriasis, arterial hypertension, gastroesophageal reflux and chronic sinusitis. He consulted for a 24-hour period characterized by headache and pain in the scalp. The examination highlights fever (38.5°), with slight erythema, sensitivity to palpation and some psoriasis plaques on the scalp. 48 hours later, it presents extensive painful erythematouspurplish plaques, present only on his right face and scalp. Biopsy was informed as Sweet Syndrome. He completed 14 days on prednisone, with excellent clinical evolution. DISCUSSION Sweet syndrome is classified into three categories: classic or idiopathic (most common), drug induced and associated with malignancy. In addition, other conditions have been described which may be related to: infections, autoimmune diseases, pregnancy. The management is focused on investigating an underlying cause and the use of corticosteroids, as a first line therapy. We report a case of idiopathic Sweet syndrome with atypical presentation, which was initially interpreted as facial cellulitis, with a rapid response to systemic corticosteroids. (AU)

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Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

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Abstract Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

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El síndrome de Sweet o dermatosis neutrofílica febril aguda es una enfermedad sistémica de etiología desconocida, caracterizada por aparición brusca de fiebre y lesiones cutáneas, asociadas con leucocitosis y neutrofilia. Puede ser idiopático o estar asociado a Enfermedades hematológicas, procesos inflamatorios, infecciones, fármacos o embarazo. Las mielodisplasias son trastornos hematológicos caracterizados por una o más citopenias secundarias a disfunción de la médula ósea. Presentamos el caso de un paciente de 81 años con síndrome de Sweet y posterior diagnóstico de síndrome mielodisplásico, con buena respuesta al tratamiento corticoideo y resolución de las lesiones cutáneas. (AU)

Sweet's syndrome or acute febrile neutrophilic dermatosis is a systemic disease of unknown etiology characterized by sudden onset of painful skin lesions; predominance of polymorphonuclear leukocytosis. It can be associated with hematologic malignancies, idiopathic, parainflammatory, secondary to drugs and pregnancy. Myelodysplasias are haematological disorders characterized by one or more cytopenias secondary to bone marrow dysfunction. We report the case of a patient of 81 years with Sweet syndrome with subsequent diagnosis of myelodysplastic syndrome with good response to corticosteroid treatment and resolution of the skin lesions. (AU)

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