Dermatosis neutrofílica de las manos y rostro posterior a mordedura de perro: reporte de caso y revisión de la literatura / Neutrophilic dermatosis of the hands and face after dog bite: case report and literature review

La Dermatosis neutrofílica de las manos es consi-derada una variante localizada acral del Síndrome de Sweet, más frecuente en mujeres y principal-mente asociada a enfermedades hematológicas. Las lesiones aparecen como pápulas, vesículas, nó-dulos, placas, úlceras y ampollas, principalmente en el dorso de las manos. Aproximadamente la mi-tad de los pacientes presenta fenómeno de patergia como factor desencadenante.En el presente caso clínico se describe una derma-tosis neutrofílica de las manos posterior a morde-dura de perro, asociado a mielofibrosis primaria y desarrollo de lesiones faciales.

Neutrophilic dermatosis of the hands is conside-red an acral localized variant of Sweet Syndrome, more frequent in women and mainly associated with hematological diseases. The lesions appear as papules, vesicles, nodules, plaques, ulcers, and blisters, mainly on the back of the hands. Appro-ximately half of the patients present a phenome-non of pathergy as a triggering factor. Herein we describe a case of neutrophilic dermatosis of the hands after a dog bite, associated with primary myelofibrosis and development of facial lesions.

Paraneoplastic Sweet's syndrome

Abstract: Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory bowel diseases, vaccination, medications and neoplasms. Hematologic malignancies are the diseases most related to paraneoplastic Sweet's syndrome, but this clinical entity can also be found occasionally in some solid tumors, including genitourinary tract tumors. We report a rare case of paraneoplastic Sweet's syndrome associated with the diagnosis of cervical cancer.

Sweet´s syndrome and pancreatic neoplasm: an atypical association

El síndrome de Sweet es una dermatosis inflamatoria poco común, que se ha asociado a tumores malignos, principalmente de tipo hematológico. Presentamos un caso clínico de síndrome de Sweet asociado con una rara neoplasia pancreática, siendo uno de los pocos casos reportados en la literatura médica acerca de esta asociación.

Sweet's syndrome is an uncommon inflammatory dermatosis, which has been associated with malignant tumors, mainly of hematological type. We report a clinical case of Sweet syndrome associated with a rare pancreatic neoplasm, which is one of the few cases reported in the medical literature about this association.

Sweet syndrome-like cutaneous drug reaction

Abstract: Cutaneous drug reactions are adverse reactions to medications that may present with different clinical features, ranging from localized to generalized lesions. In this report we describe a case of an unusual drug reaction, resembling the morphology of Sweet syndrome lesions. The patient had a psychiatric illness and was using thioridazine hydrochloride for one year. He developed infiltrated and grouped erythematous lesions on the elbows and knees three days after commencing multiple drugs (promethazine, haloperidol, mirtazapine and levomepromazine). After suspension of these four drugs and after the use of glucocorticoids, the patient had significant clinical improvement.

Sweet's syndrome associated with Crohn's disease

Abstract: Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Histiocytoid Sweet's syndrome presenting with annular erythematous plaques

Abstract Histiocytoid Sweet's Syndrome is a rare inflammatory disease described in 2005 as a variant of the classical Sweet's Syndrome (SS). Histopathologically, the dermal inflammatory infiltrate is composed mainly of mononuclear cells that have a histiocytic appearance and represent immature myeloid cells. We describe a case of Histiocytoid Sweet's Syndrome in an 18-year-old man. Although this patient had clinical manifestations compatible with SS, the cutaneous lesions consisted of erythematous annular plaques, which are not typical for this entity and have not been described in histiocytic form so far. The histiocytic subtype was confirmed by histopathological analysis that showed positivity for myeloperoxidase in multiple cells with histiocytic appearance.

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.

Sweet's syndrome associated with cellulitis - a challenging diagnosis

Abstract Sweet's syndrome is a neutrophilic dermatosis with worldwide distribution that has been associated with inflammatory autoimmune diseases, infections, malignancies, drugs, and pregnancy. The disease is idiopathic in up to 50% of patients. A 64-year-old woman, diagnosed with right limb cellulitis (4 days of evolution), was seen at our department, due to persistent cellulitis and progressive appearance of painful nodules and plaques in both shins and the right forearm (2 days of evolution). Taken together, clinical, laboratory and pathological data suggested the diagnosis of Sweet's syndrome, probably secondary to cellulitis of the right inferior limb. We suggest that cellulitis may be associated with Sweet's syndrome, a rare association in the literature.

Sweet Syndrome in childhood

AbstractSweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. It usually regresses spontaneously and treatment is primarily to control the basic disease. The authors report the case of a child of 1 year and 11 months who developed Sweet syndrome.

Concurrence of Sweet's Syndrome, Pathergy Phenomenon and Erythema Nodosum-like Lesions

We report the case of a 54-year-old woman with concurrent Sweet's syndrome, pathergy phenomenon and eythema nodosum-like lesions associated with suppurative tonsillitis. Tender, violaceous and highly edematous papules and plaques were detected on the forearms and legs, some of which were pseudovesicular. Similar edematous papules were detected on the dorsum of the hands at the sites of intravenous injection. Biopsies of a plaque on the forearm and a papule at the site of intravenous injection confirmed the presence of Sweet's syndrome. Multiple, tender, violet-red, subcutaneous erythematous nodules were revealed on the legs. A biopsy taken from a nodule on the leg revealed septal panniculitis. To our knowledge, no patient with concurrent Sweet's syndrome, pathergy phenomenon, and erythema nodosum-lesions had been reported yet in previous literature.

Síndrome de Sweet idiopático: reporte de caso / Sweet syndrome idiopathic: case report

El síndrome de Sweet (SS) es el prototipo de las dermatosis neutrofílicas, un grupo de enfermedades cutáneas de carácter reactivo caracterizadas histopatológicamente por la presencia de un infiltrado de neutrófilos. Es un marcador de diversas enfermedades internas, entre las que destacan las infecciones, la enfermedad inflamatoria intestinal, las conectivopatías autoinmunitarias y variadas neoplasias malignas, especialmente las de origen hematológico. Se presenta el caso de una mujer de 39 años, con diagnóstico de SS, tras sospecha clínica y biopsia de lesiones cutáneas.

Sweet's syndrome (SS) is the prototype of neutrophilic dermatosis, a group of skin diseases reactive in nature and characterized histopathologically by the presence of an infiltrate of neutrophils. It is a marker of various internal diseases, among which are infections, inflammatory bowel disease, autoimmune connectivopathies and various malignancies, especially those oj hematologic origin. We present a case of a 39 year old woman, diagnosed with SS after clinical suspicion and biopsy of skin lesions.

Síndrome de Sweet. Descripción de un caso idiopático / Sweet syndrome. Idiopathic case report

El síndrome de Sweet (SS) es el prototipo de las dermatosis neutrofílicas, un grupo de enfermedades cutáneas de carácter reactivo caracterizadas histopatológicamente por la presencia de un infiltrado de neutrófilos. Es un marcador de diversas enfermedades internas, entre las que destacan las infecciones, la enfermedad inflamatoria intestinal, las conectivopatías autoinmunitarias y variadas neoplasias malignas, especialmente las de origen hematológico. Se presenta el caso de una mujer de 39 años, con diagnóstico de SS, tras sospecha clínica y biopsia de lesiones cutáneas.

Sweet's syndrome (SS) is the prototype of neutrophilic dermatosis, a group of skin diseases reactive in nature and characterized histopathologically by the presence of an infiltrate of neutrophils. It is a marker of various internal diseases, among which are infections, inflammatory bowel disease, autoimmune connectivopathies and various malignancies, especially those of hematologic origin. We present a case of a 39 year old woman, diagnosed with SS after clinical suspicion and biopsy of skin lesions.

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito Santo) / Síndrome de Sweet: achados clínico-patológicos nos pacientes atendidos de 1997 a 2009 no Hospital Universitário Cassiano Antônio Moraes - Vitória (Espírito Santo)

Sweet's syndrome or acute febrile neutrophilic dermatosis is rare in Brazil. It is clinically characterized by painful erythematous nodules, papules or plaques that occur mainly on the neck and upper limbs. Its cause may be unknown (idiopathic form) or it may be associated with malignancies, usually hematologic, or drugs. The authors describe 16 cases of the syndrome. The median age was 36 years, and all patients were white and presented solid lesions, predominantly on the upper limbs and trunk. Histopathological examination of the dermis of all patients revealed predominance of moderate to intense, superficial and deep, diffuse inflammatory infiltrate, mainly consisting of polymorphonuclear neutrophils, with leukocytoclasia. It also revealed changes in the epidermis and hypodermis (neutrophilic hypodermitis), but with no signs of vasculitis in most patients, which was not considered an important finding for diagnosis. Presence of exocytosis of neutrophils was common, favoring the diagnosis of Sweet's syndrome when accompanied by diffuse interstitial neutrophilic dermatitis.

A Síndrome de Sweet ou dermatose neutrofílica febril aguda é rara no Brasil, caracterizada clinicamente por pápulas, placas ou nódulos eritematosos, dolorosos, principalmente na região cervical e membros superiores. Pode surgir sem fator desencadeante (forma idiopática) ou associar-se a neoplasias, comumente hematológicas, ou a drogas. Os autores descrevem 16 casos da Síndrome, idade mediana de 36 anos, todos da raça branca, com lesões sólidas predominantes nos membros superiores e no tronco. O histopatológico da derme observou, em todos os pacientes, predomínio de infiltrado inflamatório difuso, superficial e profundo, de moderado a intenso, composto principalmente por polimorfonucleares neutrófilos, com leucocitoclasia, além de alterações na epiderme e na hipoderme (hipodermite neutrofílica), mas sem sinais de vasculite na maioria dos pacientes, não sendo considerada achado importante para o diagnóstico. A presença de exocitose de neutrófilos foi frequente, favorecendo o diagnóstico de Síndrome de Sweet quando acompanhada por dermatite intersticial neutrofílica difusa.

Síndrome de Sweet asociado a leucemia mieloide aguda y factor estimulante de colonias de granulocitos: Caso clínico / Sweet's syndrome associated with acute myeloid leukemia: Report of one case

Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is characterized by fever, neutrophilia, erythematous and tender skin lesions that typically show a diffuse infiltrate of neutrophils in the upper dermis. This disorder has been associated with myeloproliferative syndromes. We report the case of a 53-year-old woman with an acute myeloid leukemia, presenting a Sweet's syndrome. A worsening of cutaneous lesions injuries was observed when granulocyte colony stimulating factor was added to treatment.

Síndrome de Sweet associada a linfoma de Hodgkin: relato de caso / Sweet's Syndrome associated with Hodgkin's disease: case report

A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20 por cento dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20 percent of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Síndrome de Sweet: estudo de 73 casos, com ênfase nos achados histopatológicos / Sweet's syndrome: study of 73 cases, emphasizing histopathological findings

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 por cento), brancas (49,2 por cento), entre a quarta e a sexta décadas de vida (73,8 por cento). Placas eritematosas (76,9 por cento), pápulas (43,0 por cento), pseudovesiculação (PV) (38,4 por cento) e lesões em alvo (18,5 por cento) foram as principais alterações. Entre as condições associadas, infecções de vias aéreas (15,4 por cento) e uso de drogas (10,8 por cento) foram as mais frequentes. Outras associações, representadas por um caso cada, foram: linfoma de Hodgkin, gravidez, colite ulcerativa, policitemia vera e lúpus eritematoso em paciente com Aids. Neutrófilos com leucocitoclasia (98,6 por cento), degeneração do colágeno (87,7 por cento), edema (74,0 por cento) e PV (38,4 por cento) foram os principais achados microscópicos. Eosinófilos estiveram presentes (41,1 por cento) de raros a abundantes e, em geral, não relacionados ao uso de drogas. O infiltrado inflamatório atingiu a derme profunda em 47,9 por cento das vezes e encontrou-se paniculite em 80,0 por cento dos casos nos quais a hipoderme estava representada (10 casos). CONCLUSÕES: Os achados, de modo geral, coincidem com os da literatura, destacando-se: frequente participação de eosinófilos sem correlação com a ingestão de medicamentos, paniculite e rara associação com linfoma de Hodgkin, sendo este o quinto relato de tal ocorrência, segundo o conhecimento dos autores.

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 percent), white (49.2 percent) and between 30 and 60 years of age (73.8 percent). The principal alterations found were: erythymatous plaques (76.9 percent), papules (43.0 percent), pseudo-vesiculation (PV) (38.4 percent) and target lesions (18.5 percent). With respect to the associated conditions, upper respiratory tract infections (15.4 percent) and the use of medication (10.8 percent) 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 percent), collagen degeneration (87.7 percent), edema (74.0 percent) and PV (38.4 percent). The presence of eosinophils (41.1 percent) ranged from rare to abundant and was usually unrelated to the use of medication. Inflammatory infiltrate reached the deep epidermal layer in 47.9 percent of cases and panniculitis was found in 80.0 percent 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.

Síndrome de Sweet e policondrite recidivante reveladores de síndrome mielodisplásica / Sweet's Syndrome and relapsing polychondritis signal myelodysplastic syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica.

The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.

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.

Dermatoses neutrofílicas: parte I / Neutrophilic dermatoses: part I

Os autores apresentam uma revisão das dermatoses neutrofílicas que possuem grande repercussão à saúde dos pacientes: síndrome de Sweet, pioderma gangrenoso, doença de Behçet e urticária neutrofílica. São discutidos, baseados nos resultados e conclusões de estudos relevantes publicados recentemente e na experiência dos autores, os principais aspectos clínicos, as importantes alterações histopatológicas e as opções para o manejo.

The authors present a review of neutrophilic dermatoses that have great impact on the health of patients: Sweet syndrome, pyoderma gangrenosum, Behçet's disease and neutrophilic urticaria. Major clinical aspects, histopathological changes and management options are discussed based on the results and conclusions of relevant studies recently published and on the authors' experience.