Síndrome de Sweet. Presentación de un caso y revisión de la literatura / Sweet syndrome. Case report and literature review

Se presentación el caso de una paciente femenina de 52 años con artralgias, fiebre de 39 grados, anorexia, conjuntivitis y lesiones eritemato-vesículo-pustulosas pruriginosas y dolorosas, asimétricas, en manos, brazos y espalda desde hace 3 días. En los exámenes de laboratorio los leucocitos se encontraban en 14.6 x 109 /L con 70 por ciento de neutrófilos, eritrosedimentación en 117 mm/h, la biopsia de piel evidenció dermatitis nodular y difusa, patrón Ackerman 7 concluyente para un síndrome de Sweet(AU)

We present the case of a female patient 52 year old with arthralgias, a fever of 39 degrees, anorexia, conjunctivitis, and pruritic and painful, asymmetric, erythematous-vesicular-pustular lesions on hands, arms and back for 3 days. In the laboratory tests the leukocytes were in 14.6 x 109 /L with 70 percent of neutrophils, erythrosedimentation in 117 mm / h, the skin biopsy showed nodular and diffuse dermatitis, Ackerman 7 pattern conclusive for a Sweet syndrome(AU)

Autoinflammatory skin disorders in inflammatory bowel diseases, pyoderma gangrenosum and Sweet's syndrome: a comprehensive review and disease classification criteria.

Pyoderma gangrenosum (PG) and Sweet's syndrome (SS) are skin diseases usually presenting with recurrent ulcers and erythematous plaques, respectively. The accumulation of neutrophils in the skin, characteristic of these conditions, led to coin the term of neutrophilic dermatoses to define them. Recently, neutrophilic dermatoses have been included in the group of autoinflammatory diseases, which classically comprises genetically determined forms due to mutations of genes regulating the innate immune response. Both PG and SS are frequently associated with inflammatory bowel diseases (IBDs); however, IBD patients develop PG in 1-3 % of cases, whereas SS is rarer. Clinically, PG presents with deep erythematous-to-violaceous painful ulcers with well-defined borders; bullous, pustular, and vegetative variants can also occur. SS is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions, and a diffuse neutrophilic dermal infiltrate. It is also known as acute febrile neutrophilic dermatosis. Treatment of PG involves a combination of wound care, topical medications, antibiotics for secondary infections, and treatment of the underlying IBD. Topical therapies include corticosteroids and the calcineurin inhibitor tacrolimus. The most frequently used systemic medications are corticosteroids and cyclosporine, in monotherapy or in combination. Dapsone, azathioprine, cyclophosphamide, methotrexate, intravenous immunoglobulins, mycophenolate mofetil, and plasmapheresis are considered second-line agents. Hyperbaric oxygen, as supportive therapy, can be added. Anti-TNF-α agents such as etanercept, infliximab, and adalimumab are used in refractory cases. SS is usually responsive to oral corticosteroids, and the above-mentioned immunosuppressants should be considered in resistant or highly relapsing cases.

Enfermedad de Crohn y síndrome de Sweet: una asociación infrecuente / No disponible

El síndrome de Sweet o dermatosis neutrofílica febril aguda(SS) se caracteriza por la aparición brusca de lesiones eritematosas,(pápulas, nódulos y placas) dolorosas, junto con fiebre y neutrofilia,siendo de presentación poco frecuente. Las lesiones se localizanpreferentemente en manos, brazos, parte superior deltronco, cuello y cara, con distribución asimétrica. Suele haber elevaciónde reactantes de fase aguda y en las biopsias cutáneas seidentifica una infiltración dérmica de neutrófilos sin vasculitis. Seconsidera un marcador de enfermedad sistémica en más de la mitadde los casos, asociándose a infecciones, enfermedad inflamatoriaintestinal, conectivopatías autoinmunes y diversas neoplasias.Su asociación con la enfermedad de Crohn (EC) es poco habitual,asociado sobre todo a afectación colónica. Se han publicadomenos de 50 casos en la literatura médica desde su primera descripciónen 1964, algunos de ellos simultáneos con el primer brotede la EC. Presentamos dos pacientes con enfermedad deCrohn y síndrome de Sweet diagnosticados en nuestro servicio enel momento del diagnóstico de la EC, así como su respuesta al tratamiento,evolución posterior y revisión de la literatura científica(AU)

The uncommon Sweet’s syndrome or acute febrile neutrophilicdermatosis (SS) is characterized by the sudden onset of painfulerythematous lesions (papules, nodules, and plaques) togetherwith fever and neutrophilia. The lesions are typically located onhands, arms, upper trunk, neck and face, showing an asymmetricdistribution. Acute phase reactants are usually elevated and dermalinfiltration of neutrophils without vasculitis is seen on skinbiopsies. It is considered as a marker of systemic disease in overhalf of the cases, and is associated with infections, inflammatorybowel disease, autoimmune connective tissue disorders and variousneoplasias.Its association with Crohn’s disease (CD) is unusual and it appearsmainly in association with colonic involvement. Fewer than50 cases have been published in the medical literature since itsfirst description in 1964, some concurrent with the first episodeof CD. We present two patients with Crohn’s disease and Sweet’ssyndrome diagnosed in our department at the time of CD diagnosis,as well as their response to treatment, subsequent course ofthe disease, and a review of the scientific literature(AU)

Facial Sweet's syndrome mimicking rosacea fulminans.

A 36-year-old man presented with a non-pruritic, erythematous facial rash with peri-oral and peri-orbital sparing. The initial clinicopathological diagnosis was rosacea fulminans, which was treated with 25 mg oral prednisolone and cephalexin. The patient re-presented 1 week later with exacerbation of his rash in addition to constitutional symptoms of fever and malaise. A further skin biopsy was taken and the marked neutrophilic infiltrate in the absence of vasculitis made the diagnosis of Sweet's syndrome (acute febrile neutrophilic dermatosis). High-dose prednisolone (50 mg daily), topical hydrocortisone cream and ichthammol in zinc ointment were commenced with rapid clinical improvement. This case highlights the importance of considering Sweet's syndrome as a differential diagnosis when presented with a facial eruption.

Síndrome de Sweet: revisión bibliográfica / Sweet's syndrome: a review

El síndrome de Sweet es una dermatosis caracterizada por la aparición brusca de placas eritematosas, dolorosas, predominantemente en cara, cuello y extremidades, acompañada de fiebre, leucocitosis a predominio neutrofílico y denso infiltrado dérmico leucocitario polimorfonuclear. Es bien conocida su relación con procesos neoplásicos malignos, motivo por el cuál es necesario el estudio minucioso y seguimiento del paciente. Presentamos un caso de leucemia mielomonocítica aguda asociada a ésta dermatosis, realizando una revisión bibliográfica del tema

Síndrome de Sweet: revisión bibliográfica / Sweets syndrome: a review

El síndrome de Sweet es una dermatosis caracterizada por la aparición brusca de placas eritematosas, dolorosas, predominantemente en cara, cuello y extremidades, acompañada de fiebre, leucocitosis a predominio neutrofílico y denso infiltrado dérmico leucocitario polimorfonuclear. Es bien conocida su relación con procesos neoplásicos malignos, motivo por el cuál es necesario el estudio minucioso y seguimiento del paciente. Presentamos un caso de leucemia mielomonocítica aguda asociada a ésta dermatosis, realizando una revisión bibliográfica del tema (AU)

Sweet's syndrome during treatment with all-trans retinoic acid in a patient with acute promyelocytic leukemia.

A 46 year old male with acute promyelocytic leukemia treated with all-trans retinoic acid (ATRA), developed fever, bilateral erythematous nodules in his axillary area, lower abdomen and inguinal region. Histopathologic examination of the skin biopsy revealed dense neutrophil infiltration in the dermis without vasculitis. The diagnosis of Sweet's syndrome was made. High dose methylprednisolone was administered and the lesions started to improve within 24 hours.