BERNA AKSOY, Hüseyin ÜSTÜN, Mahi BALCI, Nilgün ATAKAN

Klinik olarak nörodermatitle karışan ve romatoid artritle birlikte olan akkiz epidermolizis bülloza olgusu

Amaç: Akkiz Epidermolizis Bülloza (EBA) kronik, büllöz, bazal membran tabakasındaki anchoring fibrillere karşı gelişen otoantikorların sonucu oluşan otoimmün bir hastalıktır. Olgu sunumu: Yetmiş yaşındaki kadın hasta polikliniğimize 10 yıldır deride soyulma ve morarma şikayeti ile başvurdu. Olgumuz daha önce nörodermatit tanısıyla başarısız şekilde topikal steroid ve topikal üre içeren tedaviler kullanmıştı. Olgunun dermatolojik muayenesinde bilateral dizler, ayak bileklerinde morumsu eritemli, likenifiye, hafif skuamlı, üzerinde milium kistleri bulunan, düzensiz sınırlı, yama ve plaklar ve sağ üst kolda ve ayak parmak üzerlerinde erode, yer yer hemorajik kurutlu alanlar mevcuttu. Olgumuzun 15 yıldır romatoid artrit (RA) öyküsü mevcuttu. Hastaya histopatolojik ve klinik bulgularla EBA tanısı konuldu. Sonuç: Bu makalede kronik skar gelişimi olan ve bu skarları çeşitli merkezlerde nörodermatit olarak yorumlanarak tedavi verilen, RA’i olan bir EBA olgusu sunulmuştur.

A case of epidermolysis bullosa acquisita clinically confused with neurodermatitis and associated with rheumatoid arthritis

Objective: Epidermolysis bullosa acquisita (EBA) is a chronic, bullous, otoimmune disease resulting from autoantibodies developed against anchoring fibrils located in the basement membrane. Case Report: A 70-year- old female patient applied to the outpatient clinic with a complaint of skin peeling and purple discoloration for 10 years. She has been treated unsuccessfully with topical corticosteroids and urea preparations in the past with a diagnosis of neurodermatitis. In the dermatologic examination purplish erythematous, lichenified, slightly scaly, irregular patches and plaques containing milium cysts were present in both knees and ankles bilaterally and erosions and hemorrhagic crusts were present on her right upper arm and toes. She had a history of rheumatoid arthritis (RA) present for 15 years. A diagnosis of EBA was concluded on the grounds of clinical and histopathological findings. Conclusion: A case of EBA associated with RA, and causing chronic scar development which was interpreted and treated as neurodermatitis in different clinics is presented in this report.

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