Jeneralize Püstüler ve Artropatik Psoriazisli Bir Hastada Gastrointestinal Amiloidoz Geliþimi

Amiloidoz, amiloid adý verilen, dallanma göstermeyen lif yapýsýnda bir proteinin hücre dýþýnda birikimiyle karakterize, heterojen bir grup hastalýðý kapsar. Sekonder amiloidoz kronik inflamatuar veya infeksiyöz hastalýklarý olanlarda geliþir. Psoriazis sýk görülen inflamatuar bir deri hastalýðý olmasýna karþýn, eþlikte amiloidozun bulunmasý çok nadirdir ve literatür taramalarýna göre, bugüne dek sadece 40 olguda tanýmlanmýþtýr.
Burada 27 yaþýnda ve üç yýldýr lokalize plak tipi psoriazisi olan, daha sonra jeneralize püstüler ve spondilartropatik psoriazis geliþtiren, HLA-B27 fenotipine sahip bir erkek hasta sunulmaktadýr. Psoriazis lezyonlarýnýn yaygýnlaþmasý ve püstüler karakter kazanmasýyla birlikte hastada nefrotik sendrom ortaya çýktý ve üst gastrointestinal traktusun endoskopik biyopsilerinin histopatolojik incelemesiyle sekonder amiloidoz tanýsý kondu. Kolþisin baþlanmasýna karþýn, nefrotik sendrom hýzlý ve ilerleyici bir seyir gösterdi ve hasta tedavinin 15. gününde kaybedildi. Literatürdeki olgularýn birçoðu gibi, olgumuzda da HLA-B27 fenotipi yanýsýra psoriatik artropati mevcuttu ve bu faktörlerin amiloid birikimine yatkýnlýk oluþturduðu düþünüldü. Sunulan olguyla, nadir olmakla birlikte psoriaziste de hýzlý bir seyir gösteren, hatta ölümcül olabilen sekonder amiloidozun geliþebileceði vurgulanmaktadýr.

Gastrointestinal Amyloidosis in a Patient with Generalized Pustular and Arthropathic Psoriasis

Amyloidosis is a heterogenous group of disorders, characterized by extracellular deposition of a branching fibrillar protein, named amyloid. Secondary amyloidosis occurs in patients with chronic inflammatory or infectious processes. Although psoriasis is a common inflammatory skin disorder, associated amyloidosis is very rare and to our knowledge, has been described in only 40 cases. We describe a 29-year-old male with a HLA phenotype B27 who had had localized psoriasis for three years which progressed to generalized pustular and spondylarthropathic form. As the psoriatic lesions became generalized and pustular, the patient developed nephrotic syndrome. Secondary amyloidosis was diagnosed based on the endoscopic biopsies of the gastrointestinal tractus. Although colchicine was begun, nephrotic syndrome showed a rapid and progressive course and the patient died on the 15th day of colchicine treatment.
As the many cases in the literature, our case also showed the expression of HLA phenotype B27 and had psoriatic arthropathy, suggesting these factors to be predisposing of amyloid deposition. By the present case, we once more delineate that, though rarely, psoriasis can be complicated with secondary amyloidosis which may have a very rapid course and even fatal outcome.