Fatih BATTAL, Yusuf GÜZEL, Nazan KAYMAZ, Ezgi KIRMIZITAŞ, Şenay KURTULUŞ

Henoch Schönlein Purpurasının Gastrointestinal Tutulumuyla Seyreden Ailevi Akdeniz Ateşi Vakası

Henoch Schönlein Purpurası artrit, karın ağrısı, non-trombositopenik purpura ve böbrek tutulumu ile seyreden, çocukluk çağında en sık görülen küçük damar vaskülitidir. Palpabl purpura genelde hastalığın ilk bulgusu olarak ortaya çıkar. Ancak gastrointestinal semptomlar nadiren döküntüden önce ortaya çıkarak tanıda gecikmelere neden olabilmektedir. Bu yazıda akut batın kliniğiyle acil servisimize başvuran, apendektomi yapılan ve klinik takibi sırasında cilt tutulumunun ortaya çıkması ile Henoch Schönlein Purpurası tanısı alan bir vaka sunuyoruz. Vakamızın önemli olan bir diğer noktası da; Henoch Schönlein purpura atağı olan hastamızda yapılan genetik analizde M694V ve V726A heterozigot mutasyonu ile Ailevi Akdeniz Ateşi tanısı konmasıdır. Ailevi Akdeniz Ateşi hastalarında Henoch Schönlein Purpurası daha sık olduğu için; Henoch Schönlein Purpurası hastalarda Ailevi Akdeniz Ateşi bulguları sorgulanmalı ve Ailevi Akdeniz Ateşi bulguları belirtilmişse gen mutasyon analizi yapılmalıdır.

Familial Mediterranean Fever Present with Gastrointestinal Manifestations of Henoch Schonlein Purpura

Henoch Schönlein Purpura is the most common small vessel vasculitis in childhood which is characterized with non-thrombocytopenic purpuric skin rash, arthritis, abdominal pain and renal disease. Palpable purpura usually occurs as the first clinical finding. However, gastrointestinal symptoms can rarely appear before the rash and may cause delays in diagnosis. Here, we present a case of Henoch Schönlein Purpura who underwent appendectomy with the diagnosis of acute abdomen and developed skin involvement in clinical follow-up. Another important point of our case is; In the genetic analysis performed in our patient with Henoch Schönlein purpura attack, Familial Mediterranean Fever was diagnosed with heterozygous mutation M694V and V726A . Since Henoch Schönlein Purpura is more common in Familial Mediterranean Fever patients; Familial Mediterranean Fever findings should be questioned in patients with Henoch Schönlein Purpura and gene mutation analysis should be performed if Familial Mediterranean Fever findings are specified.

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