Pustulosis subcórnea: a propósito de un caso / Subcorneal pustulosis: a case report

Resumen La dermatosis pustulosa subcórnea (DPS), oenfermedad de Sneddon-Wilkinson, es una dermatosis poco frecuente, benigna, de curso crónico y recidivante. En esta oportunidad presentaremos el caso de una paciente femenina de 58 años condiagnóstico de dermatosis pustulosa subcórnea, que fue tratada con dapsona 100mg por día, con resolución de lesiones. Realizamos unarevisión bibliográfica de esta patología describiendo sus características clínicas e histológicas, sus múltiples diagnósticos diferenciales y las posibles terapéuticas.

Abstract Subcorneal pustular dermatosis, or Sneddon-Wilkinson disease, is a rare, benign, chronic and recurrent dermatosis. We reportthe case of a 58-year-old female patient with subcorneal pustular dermatosis, who was treated with dapsone 100mg per day, with goodtherapeutic response. We carried out a bibliographic review of this pathology describing its clinical and histological characteristics, its multiple differential diagnoses and possible therapeutic options.

Síndrome de SWEET. A propósito de 1 caso / SWEET syndrome. About 1 case

El Síndrome de Sweet llamado también dermatosis neutrofílica febril aguda es una enfermedad rara, de naturaleza inflamatoria, caracterizada por fiebre de inicio agudo, neutrofilia, lesiones cutáneas eritematosas y dolorosas, infiltrado típico de neutrófilos en la dermis superior y rápida mejoría con corticoesteroides sistémicos. Presenta formas típicas y atípicas, las primeras cumplen con todos los criterios de diagnóstico y las causas pueden ser neoplásica, infecciosa, fármacos, embarazo y a veces idiopática. Se presenta un caso de Síndrome de Sweet típico en una mujer, probablemente desencadenado por un cuadro infeccioso de vías aéreas superiores con confirmación histológica y buena respuesta a la corticoterapia, con remisión total y sin recidiva a la fecha

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare, inflammatory disease characterized by acute-onset fever, neutrophilia, erythematous and painful skin lesions, a typical neutrophil infiltrate in the upper dermis, and rapid improvement with systemic corticosteroids. It presents typical and atypical forms, the former meet all diagnostic criteria and the causes can be neoplastic, infectious, drugs, pregnancy, and sometimes idiopathic. We present a case of typical Sweet syndrome in a woman, probably triggered by an infectious picture of the upper airways with histological confirmation and good response to corticosteroid therapy, with total remission and no recurrence to date

Neutrophilic dermatosis of the dorsal hands related to cocaine abuse

Abstract Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

lgA pemphigus, intraepidermal neutrophillic type in an 8-year old Filipino female: A case report

INTRODUCTION@#lgA pemphigus is a rare, chronic, relapsing, benign group of autoimmune intraepidermal blistering dermatosis with an unknown etiology. It is characterized by significantly pruritic, vesiculopustular lesions that occur mainly on the trunk and proximal extremities. Histopathologic and immunofluorescence studies show intraepidermal blisters and deposition of immunoglobulin A in the intercellular spaces of the epidermis, respectively.@*CASE REPORT@#To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone.

Pyoderma Gangrenosum Arising De Novo Over an Unusual Site: A Case Report

Summary@#Pyoderma gangrenosum (PG) of the breast is a rare rapidly progressive neutrophilic dermatosis, which usually co-exists with severe underlying systemic conditions. A woman presented with a non-healing ulcer over her right breast with characteristic sparing of nipple-areola complex (Bork-Baykal phenomenon). It was diagnosed as pyoderma gangrenosum on the basis of clinico-pathological correlation and managed successfully with systemic corticosteroids and anti-inflammatory drugs along with wound care. The diagnosis and treatment of PG is challenging particularly at unusual sites given the paucity of robust clinical evidence and lack of consensus opinion regarding specific management guidelines. It is imperative that PG is considered as a clinical diagnosis in any patient with enlarging, sterile, necrotic lesions unresponsive to appropriate antibiotics. Early recognition of PG at rare locations can prevent devastating sequelae such as over-zealous surgical debridement and deep tissue infections associated with a chronic open wound leading to severe cosmetic morbidity.

Pioderma gangrenoso como manifestación Inicial de síndrome mielodisplásico / Pyoderma gangrenosum as initial manifestation of syndrome myelodysplastic

El pioderma gangrenoso es una dermatosis neutrofílica poco común que se presenta como un desorden inflamatorio y ulcerativo de la piel. Más de la mitad de los pacientes que desarrollan esta afección, tienen asociada una enfermedad sistémica subyacente. Caso clínico: paciente femenina de 79 años de edad, previamente sana, quien acude por presentar desde 4 días antes, flictenas violáceas, de contenido serohemático que se decapitan dejando úlcera superficial de fondo eritematoso, y luego profundizaron, no mejoraron con antibióticos por lo que se toma biopsia cutánea que reporta hallazgos compatibles con pioderma gangrenoso; se inició tratamiento con corticoides tópicos y sistémicos evidenciando mejoría de las lesiones. Durante la investigación etiológica se constató descenso de todas las líneas hematológicas y la biopsia de médula ósea mostró síndrome mielodisplásico con displasia unilínea de bajo grado. Discusión: El pioderma gangrenoso de tipo buloso, está comúnmente asociado a enfermedades hematológicas; en pacientes sin alteraciones hematológicas iniciales, debe haber un seguimiento estricto enfocado en la búsqueda de estos trastornos en un lapso de hasta 10 años. Se trata con fármacos inmunosupresores e inmunomoduladores. Conclusiones: ante la presencia de pioderma gangrenoso se debe sospechar síndrome mielodisplásico(AU)

Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin. More than half of patients with pyoderma gangrenosum develop the disorder in association with an underlying systemic disease. Clinical case: 79-year-old female patient, previously healthy, who consulted for a period of 4 days, purplish skin lesions, which, once decapitated, showed a superficial ulcer that did not improve with broad spectrum antibiotics. The biopsy is taken, which reports findings compatible with pyoderma gangrenosum, so treatment with topical and systemic corticosteroids is started showing improvement of the lesions. During the diagnostic workup, pancytopenia was verified and, after ruling out other etiologies, bone marrow biopsy was performed, which allowed the diagnosis of lower-risk myelodysplastic syndrome with single lineage dysplasia. Discussion: Bullous pyoderma gangrenosum is most commonly seen in patients with hematologic disease; due to the strong association between bullous PG and hematologic disease, patients who present without an associated hematologic disorder should be followed closely for the development of a hematologic disorder. Conclusion: Etiological search for pyoderma gangrenosum allows early diagnosis and timely treatment of the underlying disease(AU)

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.

Síndrome de Sweet en una niña preescolar / Sweet syndrome in a 5-year-old girl

El síndrome de Sweet, también conocido como dermatosis neutrofílica febril, es un trastorno dermatológico poco frecuente en pediatría. Clínicamente, se caracteriza por la aparición de lesiones papulares y/o nodulares de una coloración rojiza-violeta con hipersensibilidad local. Se reporta el caso de una paciente femenina de 5 años, quien consultó por un cuadro clínico de 10 días de evolución de aparición de lesión forunculosa en el arco nasal. Se realizó una biopsia de piel, que reportó dermatitis difusa con predominio de polimorfonucleares neutrófilos, necrosis epidérmica y ausencia de vasculitis. No se identificaron microorganismos. Se consideró el cuadro compatible con síndrome de Sweet. Es importante tener en cuenta este diagnóstico en cuadros clínicos similares y se deben descartar otros diagnósticos más frecuentes primero.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is an infrequent dermatological disorder in pediatrics. Clinically it is characterized by the development of papular and/or nodular lesions of a reddish-violet coloration with local hypersensitivity. We report the case of a 5-year-old female who consulted 1 month after the appearance of the lesion in the nasal arch. A skin biopsy was performed and it reported diffuse dermatitis with a predominance of neutrophil polymorphonuclear cells, epidermal necrosis and absence of vasculitis. No microorganisms were identified. It was considered compatible with Sweet syndrome. It is important to consider this diagnosis in similar clinical cases and other more frequent diagnoses must be ruled out first.

Síndrome de Sweet en pediatría: a proposito de un caso / Sweet syndrome in pediatrics: a case report

INTRODUCCIÓN: El Síndrome de Sweet es una dermatosis muy infrecuente en pediatría, de patogenia desconocida, caracterizado clínicamente por fiebre, neutrofilia, placas solevantadas y dolorosas en piel de cara, cuello y extremidades e histológicamente por infiltración dérmica de neutrófilos. OBJETIVO: Presentación de un caso clínico de Síndrome de Sweet en paciente pediátrico. CASO CLÍNICO: Prees colar femenino de 3 años, portador de cromosomopatía compleja 46XX add(8). Consultó por cuadro clínico de 7 días de evolución caracterizado por placas y ampollas localizadas en dorso y posterior mente en extremidades, asociado a fiebre alta, sin respuesta a tratamiento antibiótico indicado por sospecha de impétigo ampollar. Al examen físico presentaba múltiples placas eritemato-violáceas, con centro ampollar en dorso, extremidades superiores e inferiores, junto a placas y nódulos eritematosos en brazo y muslo izquierdo. En los exámenes de laboratorio destacó leucocitosis con neutrofilia (RAN 45954/mm3) y PCR elevada (347 mg/L). Se realizó biopsia de lesiones cutáneas con hallazgos histopatológicos compatibles con Síndrome de Sweet. Se indicó tratamiento con prednisona 1 mg/ kg/día, con buena respuesta clínica. A las 2 semanas de tratamiento presentaba placas costrosas de menor tamaño, sin lesiones ampollares. CONCLUSIONES: El síndrome de Sweet corresponde a una dermatosis infrecuente en pediatría, por lo que se debe tener un alto índice de sospecha ante la presencia de fiebre asociado a lesiones cutáneas persistentes. Si bien la mayoría de los casos son idiopáticos, se debe realizar el tamizaje de condiciones asociadas, principalmente de trastornos proliferativos, infecciones e inmunodeficiencias.

INTRODUCTION: Sweet's syndrome is a very rare dermatosis in pediatrics, of unknown pathogenesis, clinically characterized by fever, neutrophilia, raised and painful plaques on the skin of the face, neck, and limbs, and histologically by dermal infiltration of neutrophils. OBJECTIVE: To present a clinical case of Sweet Syndrome in a pediatric patient. CLINICAL CASE: 3-years-old female child, with history of complex chromosomopathy 46XX add(8), with a 7-day history of plaques and blisters on the back and later also on the limbs, associated with high fever, without response to antibiotic treatment pres cribed due to suspicion of bullous impetigo. Physical examination showed multiple erythematous- violaceous plaques, with bullous center on the back, upper and lower limbs, along with plaques and erythematous nodules on the left arm and thigh. Laboratory tests showed leukocytosis with neutro philia (absolute neutrophil count 45954/mm3) and elevated CRP (347 mg/L). Biopsy of skin lesions reported histopathological findings compatible with Sweet's Syndrome. Treatment with prednisone 1 mg/kg/day was indicated with good clinical response. After two weeks of treatment, she presented crusty plaques of smaller size, without bullous lesions. CONCLUSIONS: Sweet's syndrome is an uncom mon dermatosis in pediatrics, therefore, a high index of suspicion should be held in the presence of fever associated with persistent skin lesions. While most cases are idiopathic, screening for associated conditions, mainly proliferative disorders, infections, and immunodeficiencies must be performed.

Sweet's Syndrome Associated with Graves' Disease

Sweet's syndrome, or acute febrile neutrophilic dermatosis, occurs in association with autoimmune diseases such as Hashimoto's thyroiditis but is rare in Graves' disease, in which all cases are induced by propylthiouracil (PTU). We report a case of Sweet's syndrome in a patient with Graves' disease treated with methimazole (MMI) during three weeks. A 34-year-old man presented with the acute onset of high fever, skin rashes on the whole body, arthralgia, and acroparesthesia. Laboratory results showed leukocytosis and elevated C-reactive protein. MMI first stopped and antibiotics and antihistamine therapy started, but his symptoms dramatically improved after oral prednisolone. Graves' disease has again been treated by MMI because of his aggravated ophthalmopathy. After one year of retreatment with MMI, there has been no recurrence of Sweet's syndrome, supporting that Sweet's syndrome in this case was not related to MMI exposure. To our knowledge, this is the first report of Sweet's syndrome associated with Graves' disease per se but not PTU or MMI use.

A Case of Steroid-resistant Neutrophilic Dermatosis of the Hands Treated with Dapsone / 대한피부과학회지

No abstract available.

A Case of Steroid-resistant Neutrophilic Dermatosis of the Hands Treated with Dapsone / 대한피부과학회지

No abstract available.

Typical Sweet Syndrome at the Site of Postmastectomy Lymphedema: Two Cases and a Literature Review / 대한피부과학회지

Sweet's syndrome (SS) or acute febrile neutrophilic dermatosis has been reported to occur in association with many conditions. We report two cases of SS at the site of a postmastectomy lymphedema. A localized variant of SS restricted to an area of postmastectomy lymphedema is an often unrecognized complication of breast cancer. Moreover, attempts to treat SS using various methods might prolong the duration of treatment. Therefore, SS should not be confused with other disorders such as erysipelas, cellulitis, herpes zoster, and contact dermatitis.

Non-bullous Neutrophilic Dermatosis in a Patient with Lupus Nephritis / 대한피부과학회지

Neutrophilic infiltrates of skin in the setting of lupus patients with bullous systemic lupus erythematosus (SLE) and leukocytoclastic vasculitis are well documented. However, most dermatology studies do not consider cutaneous involvement by SLE in the histologic differential diagnosis of neutrophilic inflammatory dermatosis in non-bullous or non-vasculitic lesions. Nevertheless, an increasing number of studies have reported cell-rich or Sweet's-like neutrophilic reactions as well as patients with skin lesions characterized by paucicellular neutrophilic dermal infiltrates. A 56-year-old female patient with history of lupus nephritis presented with multiple erythematous to brownish papulo-patches and plaques on both legs for 1 month. She had no signs or systemic symptoms of Sweet's syndrome. Histopathologic finding showed perivascular and interstitial neutrophilic infiltration with leukocytoclasia in the upper dermis, but not as dense as commonly observed in Sweet's syndrome with absence of papillary dermal edema. Herein, we present a rare case of non-bullous neutrophilic dermatosis associated with SLE. It is important to consider SLE-associated neutrophilic dermatosis in the differential diagnosis of neutrophilic tissue reactions and to be aware of the broad histologic spectrum that may be encountered in SLE-associated neutrophilic dermatosis, ranging from subtle paucicellular lesions to florid Sweet's-like lesions.

A Case of Neutrophilic Dermatosis of the Hands on Both Palms / 대한피부과학회지

Neutrophilic dermatosis of the hands is a rare, peculiar variant of acute febrile neutrophilic dermatosis (Sweet syndrome). It initially appears on the hands and possibly spreads to other locations. Clinically, neutrophilic dermatosis of the hands tends to occur as tender erythematous plaques, pustules, and bullae that are limited to the dorsal hands and fingers. Histopathologically, vasculitis has been observed in pustular lesions on the dorsal hands. Therefore, the terms "pustular vasculitis of the dorsal hands" and "neutrophilic dermatosis of the dorsal hands" were suggested initially. Here, we report a case of neutrophilic dermatosis of the hands in a 77-year-old woman who presented with painful well-demarcated plaques and macules on both palms without dorsal hand involvement. Histopathological findings showed leukocytoclastic vasculitis in the erythematous lesion on the palm.

A Case of Neutrophilic Dermatosis of the Dorsal Hands / 대한피부과학회지

No abstract available.

Síndrome de Sweet y embarazo: a propósito de un caso / Sweet syndrome and pregnancy: case report

El síndrome de Sweet, también conocido como dermatosis neutrofílica febril aguda, es una entidad infrecuente, de etiología desconocida, caracterizada por la aparición brusca de placas eritematosas, dolorosas, acompañadas de fiebre, leucocitosis neutrofílica y denso infiltrado dérmico leucocitario polimorfonuclear. Si bien la mayoría de los casos son idiopáticos, es bienconocida su relación con procesos neoplásicos malignos, enfermedades autoinmunes, inflamatorias, infecciosas, fármacos y embarazo. Presentamos el caso de una paciente con síndrome de Sweet asociado a embarazo y realizamos una revisión bibliográfica de esta patología.

Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is a rare entity of unknown etiology characterized by the sudden onset of painful erythematous plaques, associated with fever, leukocytosis with neutrophilia as well as a dense dermal polymorphonuclear leukocytic infiltrate. Although most cases are idiopathic, it is well known to be related with malignant neoplastic processes, autoimmune, inflammatory, infectious diseases, drugs and pregnancy. A case of a pregnant woman with Sweet ́s syndrome diagnosis is reported and a literature review is performed.

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 localizado sobre linfedema: descripción de un caso y revisión de la literatura / Sweet´s syndrome located on lymphoedema: a case report and review of literature

Comunicamos el caso de una mujer de 63 años de edad, que desarrolló lesiones compatibles con un síndrome de Sweet, localizadas en el miembro sometido a linfadenectomía e irradiación, debido a una neoplasia maligna neural. Efectuamos comentarios del caso y los comparamos con la bibliografía publicada.

A 63 years-old woman treated wit lymphadenectomy and radiotherapy due a malignant neural neoplasia, developed lesions of a Sweet's syndrome, localized in the area treated. A review and comparative study of the previous papers are made.