Neutrophilic dermatosis of the dorsal hands: response to dapsone monotherapy

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.

Síndrome de Sweet: revisión de la literatura a propósito de un caso / Sweet syndrome: review of the literature about a case

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)

Type 2 leprosy reaction with Sweet's syndrome-like presentation

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.

Síndrome de Sweet asociado a síndrome mielodisplásico / Sweet syndrome associated with myelodysplastic syndrome

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)

Sweet’s syndrome.

Acute febrile neutrophilic dermatosis or Sweet’s syndrome (SS) is characterized by painful, erythematous plaques of rapid onset accompanied by fever. The etiology of SS is unknown and it may be associated with antecedent infections, malignancies, autoimmune diseases, drugs and vaccines, upper respiratory or gastrointestinal infection, pregnancy, inflammatory bowel disease as well as chemotherapy or idiopathic. The standard therapy for SS is systemic corticosteroids. We report a rare case of 19‑year‑old young male patient with complaint of severe ill‑defined type of pain in both jaws associated with plaques and papules on extensor surfaces of upper and lower extremities with bodyache and myalgia. Histopathological examination suggested perivascular neutrophilic infiltration with scattered eosinophils. Sweet syndrome has rare oral manifestations secondary to hematological changes. It can also present as a paraneoplastic syndrome (malignancy‑associated form of condition, which is most commonly related to acute myelogenous leukemia), which leads to poor prognosis and thus it requires careful examination, early diagnosis and long‑term follow‑up.

Síndrome de Sweet: estudo de 23 casos / Sweet's syndrome: a study of 23 cases

FUNDAMENTOS: A síndrome de Sweet (SS) é uma doença rara, caracterizada por lesões cutâneas eritematovioláceas dolorosas, febre, leucocitose com neutrofilia e derme com infiltrado inflamatório neutrofílico denso à histologia. Apresenta excelente resposta à corticoterapia. OBJETIVOS: Avaliar os casos de SS em hospital universitário, identificando as características clínicas, laboratoriais e epidemiológicas e compará-las com os dados da literatura. MÉTODOS: Realizou-se estudo epidemiológico, retrospectivo, mediante revisão de prontuários. Identificaram-se 23 pacientes que preencheram os critérios diagnósticos para a doença no período de março de 1995 a julho de 2009. Coletaram-se dados clínicos e epidemiológicos dos pacientes, tais como: localização das lesões, presença de manifestações cutâneas e extracutâneas, condições associadas à SS e alguns dados laboratoriais, como con tagem de leucócitos e velocidade de hemossedimentação (VHS). RESULTADOS: As idades variaram entre 2 e 75 anos. Houve predomínio do sexo feminino. As lesões acometeram, preferencialmente, tronco e membros superiores. Febre foi a manifestação sistêmica mais comum, seguida por artralgias e mialgia, conjuntivite e artrite. Os fatores desencadeantes mais comumente detectados foram infecções de vias aéreas. Neoplasias associadas ocorreram em 30 por cento dos pacientes, principalmente hematológicas. CONCLUSÕES: Os dados clínicos e epidemiológicos encontrados no presente estudo são, em sua maior parte, similares aos já disponíveis na literatura. Devido à alta prevalência de doenças malignas na SS é importante diagnosticá-la, realizar investigação sistêmica adequada e manter seguimento dos pacientes.

BACKGROUND: Sweet's syndrome or acute febrile neutrophilic dermatosis is a rare disease characterized by painful violaceous erythematous skin lesions, fever, neutrophilic leukocytosis and dense dermal neutrophilic inflammatory infiltrate. It shows excellent response to corticosteroids. OBJECTIVES: To assess cases of Sweet's syndrome in a university hospital, identifying its clinical, laboratory and epidemiological characteristics and compare them with the data found on the literature. METHODS: We conducted a retrospective epidemiological study by examining the medical records of 23 patients who met the diagnostic criteria for the disease from March 1995 to July 2009. We collected clinical and epidemiological data on the patients, such as lesion location, presence of cutaneous and extracutaneous manifestations, conditions associated with SS and some laboratory data, such as leukocyte count and ESR. RESULTS: The age of the patients in the study ranged from 2 to 75 years. There were more females. The lesions mostly affected the trunk and upper limbs. Fever was the most common systemic manifestation, followed by arthralgia and myalgia, conjunctivitis and arthritis. The triggering factors most commonly identified were infections of the respiratory tract. Associated neoplasia occurred in 30 percent of the patients, especially hematologic neoplasia. CONCLUSION: The clinical and epidemiological data found in our study are mostly similar to those found in the literature. Given the high prevalence of malignant diseases in patients with Sweet's syndrome, it is necessary to know how to perform the diagnosis, carry out a full investigation as well as do the patient's follow up.

Sweet's syndrome

Sweet's syndrome [acute febrile neutrophilic dermatosis] is characterized by classical skin lesions accompanied by fever and malaise. Systemic involvement may be present and lung involvement in Sweet's syndrome has been reported in the form of bilateral pulmonary infiltrates,bronchiolitis obliterans organizing pneumonia and pleural effusion. There are dense papillary neutrophilic infiltrates on histopathology. We present a case of Sweet's syndrome with right lower lobe consolidation and persistent fever which was non-responsive to antibiotics but showed clinical improvement with clearing of radiological opacities on oral steroid therapy

Dermatosis neutrofílica del dorso de las manos: presentación de un caso y revisión de la literatura / Neutrophilic dermatosis of the dorsal hands

La dermatosis neutrofílica del dorso de las manos es una nueva entidad dermatológica, descrita por Strutton y col. en 1995. Parecería ser una variante localizada del síndrome de Sweet, caracterizada por una erupción cutánea restringida al dorso de las manos y/o de los dedos y que histopatológicamente presenta un infiltrado neutrofílico con o sin vasculitis. Responde adecuadamente al tratamiento con corticoides y dapsona. Se presenta una paciente de 59 años con antecedente de colitis ulcerosa, que consultó por una placa de 3 cm de diámetro, dolorosa, de superficie erosiva, de 3 semanas de evolución. Luego del tratamiento local con antisépticos, la lesión curó espontáneamente, sin recidivas hasta la fecha. El diagnóstico definitivo de dermatosis neutrofílica del dorso de las manos se hizo en base a la clínica, la biopsia y la evolución. Se hace una exhaustiva revisión de la literatura que incluye 19 casos publicados, a los cuales le agregamos el nuestro

Síndrome de Sweet: presentación de un caso / Sweet's syndrome: case review

Se presenta un caso clínico de síndrome de Sweet diagnosticado en una paciente de 31 años. El diagnóstico se estableció sobre las bases clínicas e histológicas. El tratamiento consistió en corticoides sistémicos, con buena respuesta. Se aprovecha para revisar parte de la bibliografía existente en relación con la etiopatogenia, clínica, asociación a otras enfermedades y tratamiento

Sindrome de Sweet a propósito de 10 casos / Sweets syndrome 10 case study

FUNDAMENTO - A síndrome de Sweet(SS) é uma doença rara, inflanmatória, de causa desconhecida que requer avaliaçäo médica e investigaçäo. OBJETIVO - Identificar os achados clínico-epidemiológicos e laboratoriais dos pacientes com SS e comparar os resultados obtidos com os da literatura. PACIENTES E MÉTODOS - Foi realizado um estudo retrospectivo de 10 pacientes com diagnóstico de SS no ambulatório de dermatologia da Universidade Federal do Paraná no período de março de 1995 a abril de 1997. Foram analisados os dados epidemiológicos, manifestaçöes cutâneas e sistêmicas, assim como condiçöes associadas à SS nesses pacientes. Foram também avaliadas análises laboratoriais, incluindo contagem de leucócitos e velocidade de hemossedimentaçäo(VHS). RESULTADOS - Foi observada uma erupçäo cutânea típica de placas eritematosas e dolorosas em 8 dos 10 pacientes. A manifestaçäo sistêmica mais freqüente foi febre. A avaliaçäo laboratorial mostrou neutrofilia em sete pacientes e taxa elevada da VHS em nove. Ocorreram condiçöes associadas em oito pacientes, sendo encontrada malignidade em seis, dos quais cinco apresentaram neoplasia hematológica, e um, carcinoma da tireóide. Outras associaçöes incluíram: retocolite ulcerativa (um paciente) e infecçäo das vias aéreas superiores (um paciente). CONCLUSÄO - Malignidade associada à SS foi um achado freqüente nos pacientes. Assim, a importância do diagnóstico da SS e da pesquisa para neoplasia deve ser enfatizada nessa entidade, que deveria ser considerada um marcador cutâneo de doença sistêmica.

A case of Sweet's syndrome in patient with dermatomyositis

Sweet's syndrome (SS) has been reported as an association with malignant neoplasms and autoimmune diseases, e.g., Behcet's disease, Sjogren's syndrome, and rheumatoid arthritis. But dermatomyositis (DM), one of the rare autoimmune diseases, was not reported as an associated disease of SS. We describe an interesting case of SS associated with DM. Diagnosis was made by skin biopsy, and subsequent clinical resolution occurred after institution of prednisolone.

Síndrome de Sweet y eritema nodoso / Sweet's syndrome and erythema nodosum

El síndrome de Sweet y el eritema nodoso representan dermatosis reactivas a diversos estímulos o trastornos asociados, algunos de los cuales han sido involucrados en el origen de ambos procesos por separado. Por este motivo, su concurrencia no debe ser considerada una asociación fortuita, sino más bien como diferentes respuestas cutáneas a un factor precipitante común. Presentamos 3 mujeres con aparición simultánea de síndrome de Sweet y eritema nodoso. En dos de ellas, ambas dermatosis ocurrieron tras una infección de la vía aérea superior, respondiendo rápidamente a prednisona oral. La tercera presentaba una celulitis bacteriana asociada que fue tratada exitosamente con cloxacilina; ésto se acompañó de una remisión completa de las lesiones de síndrome de Sweet, conjuntamente con las de eritema nodoso

Informe de un caso de síndrome de Sweet asociado a tumores sólidos / Report of a Sweet's syndrome associated to a solid tumor

El síndrome de Sweet (SS) ha sido ocasionado con neoplasias hematológicos y en menor grado con tumores sólidos; la patogénesis aún no se ha dilucidado, sin embargo, hay evidencias a favor de mecanismos inmunopatogénicos. Se presenta un caso clínico de un paciente con SS y se revisan los factores que actualmente han sido involucrados en su patogenia

Dermatosis neutrofílica: serie clínica / Neutrophilic dermatoses: clinical serial

Las dermatosis neutrofílicas comprenden una serie de enfermedades que histológicamente comparten un infiltrado de neutrófilos prominente y que parecen tener un mecanismo patogénico común. En este artículo presentamos una serie clínica de pacientes con esta dermatosis y se discuten algunos aspectos clínicos, histopatológicos y patogénicos de relevancia