Sweet’s syndrome – report of a patient with a variety of extraintestinal manifestations of ulcerative colitis

Abstract

Sweetov sindrom je rijedak kožni poremećaj kojeg karakterizira izbijanje bolno osjetljivih crvenkastih plakova po koži, što je često praćeno febrilitetom, neutrofilijom u perifernoj krvi te nalazom pretežno perivaskularno smještenih nakupina neutrofila u histološkom preparatu bioptata kože. Bolest je u 50% slučajeva povezana s drugim bolestima i stanjima kao što su maligne bolesti, bakterijske i virusne infekcije, autoimune bolesti i kolagenoze, upalne bolesti crijeva, upotreba nekih lijekova, itd. Ulcerozni kolitis je idiopatska, imunološki uvjetovana upalna bolest crijeva koja je u malog broja bolesnika praćena i vancrijevnim manifestacijama bolesti, koje se najčešće razvijaju na koštano-zglobnom sustavu, koži i sluznicama. U ovom radu prikazujemo 39-godišnju bolesnicu s ulceroznim kolitisom u koje su se šest mjeseci nakon pojave kolitičkih tegoba razvili Sweetov sindrom, reaktivni poliartritis i poliserozitis. Kombiniranim protuupalnim liječenjem metil-prednizolonom i mesalazinom u terapijskim dozama postignuta je remisija ulceroznog kolitisa i povlačenje svih simptoma vancrijevnih manifestacija bolesti, no tri mjeseca kasnije je u bolesnice došlo do relapsa reaktivnog poliartritisa, koji je uspješno suzbijen primjenom ibuprofena. Kod sumnje na Sweetov sindrom potrebno je učiniti histološki pregled kožnih promjena te ukoliko se uz karakterističan histološki nalaz i prisutne ostale kliničke i laboratorijske kriterije postavi dijagnoza Sweetovog sindroma, potrebno je tragati za predležećim bolestima. Kao zlatni standard preporučeno liječenje sistemskom primjenom kortikosteroida se pokazalo učinkovitim u liječenju naše bolesnice.Sweet’s syndrome is a rare dermatological disorder characterized by eruption of painful reddish plaques on the skin, accompanied by fever, neutrophilia in peripheral blood and finding of perivascular neutrophilic infiltrates in the skin biopsy sample. The syndrome is in 50% of cases associated with other diseases and conditions such as malignancies, bacterial and viral infections, autoimmune and connective tissue diseases, inflammatory bowel diseases, use of certain drugs, etc. Ulcerative colitis is idiopathic, immunologically mediated inflammatory bowel disease which is in a small proportion of patients associated with extraintestinal manifestations of disease, characteristically developed on the joints, skin and mucous membranes. We present a case of a 39-year-old woman with ulcerative colitis in whom the six month period of colitis symptoms was followed by the development of Sweet’s syndrome, reactive polyarthritis and polyserositis. Using the combined anti-inflammatory treatment with methylprednisolone and mesalazine in therapeutical doses the remission of ulcerative colitis and regression of all extraintestinal manifestations was achieved, but three months later a relapse of reactive polyarthritis developed that was successfully treated with ibuprofen. When Sweet’s syndrome is suspected, the histological examination of the skin lesions should be performed, and if a characteristic histological finding is accompanied by other clinical and laboratory criteria and the diagnosis of Sweet’s syndrome is established, the search for underlying diseases should be done. The systemic treatment with corticosteroids, recommended as the golden standard, proved effective in the treatment of our patient