Crohn's disease and Sweet's syndrome: an uncommon association.

Sweet's syndrome or acute febrile neutrophilic dermatosis (SS) is characterized by the sudden onset of painful erythematous lesions (papules, nodules, and plaques) together with fever and neutrophilia. The lesions are typically located on hands, arms, upper trunk, neck and face, showing an asymmetric distribution. Acute phase reactants are usually elevated and dermal infiltration of neutrophils without vasculitis is seen on skin biopsies. It is considered as a marker of systemic disease in over half of the cases, and is associated with infections, inflammatory bowel disease, autoimmune connective tissue disorders and various neoplasias. Its association with Crohn's disease (CD) is unusual and it appears mainly in association with colonic involvement. Fewer than 50 cases have been published in the medical literature since its first description in 1964, some concurrent with the first episode of CD. We present two patients with Crohn's disease and Sweet's syndrome diagnosed in our department at the time of CD diagnosis, as well as their response to treatment, subsequent course of the disease, and a review of the scientific literature.

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)