Çocukluk çağı mastositoz olgularımız: tek merkez sonuçları

Amaç: Kliniğimizde tanı alan ya da sistemik tutulum açısından diğer bö- lümlerden kliniğimize yönlendirilen mastositoz olgularının klinik, demografik, histopatolojik özellikleri, hastalık seyri ve tedavi sonuçlarının değerlendirilmesi amaçlandı. Gereç ve Yöntemler: İki bin-2014 yılları arasında, mastositoz tanısı alan 21 olgunun dosyası geriye dönük olarak değerlendirildi. Bulgular: Olguların tümünde kutanöz mastositoz vardı; 19u ürtikerya pigmentoza, ikisi mastositoma idi. Erkek kız oranı: 1/1,6 idi. Olgularda yakınmaların ortanca görülme yaşı 12,1 ay idi; üç olguda yakınmalar yenidoğan döneminde başlamıştı. En sık görülen yakınma olan döküntü tüm olgularda vardı; 10 olguda kaşıntı, bir olguda bül oluşumu, bir olguda karın ağrısı ve bir olguda ataklar şeklinde tüm bedende kızarma ve gö- ğüste yanma hissi saptandı. Aile öyküsü iki olguda pozitifti. Olguların tü- münde tanı deri biyopsisi ile doğrulandı. Olguların ortanca izlem süresi beş yıl idi. Tedavide yakınmalara yönelik olarak H1 ve H2 antihistaminik, yerel nemlendirici ve yerel kortikosteroidli ilaçlar kullanıldı. İzlemde ergenlik dönemine ulaşan dört olgunun tümünde lezyonlar kendiliğinden tamamen düzeldi, yedi olguda döküntüler ortalama 5,5 yıl izlem süresi içinde azalırken, ortalama 3,6 yıl izlenen 10 olguda lezyonların aynı şekilde kaldığı saptandı. Çıkarımlar: Mastositoz çocukluk çağı döküntüleri arasında akla gelmesi gereken bir tanıdır. Çocukluk çağında sıklıkla kutanöz mastositoz görü- lür. Sistemik tutulum nadirdir. Hastalık kendini sınırlar ve ergenlik dö- neminde kaybolma eğilimindedir.

Childhood mastocytosis: results of a single center

Aim: We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis who were referred to our clinic for evaluation of systemic involvement and/or who were diagnosed with mastocytosis in our clinic. Material and Methods: The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed. Results: All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1.6. The median age for onset of disease was 12.1 months and the disease occured in the newborn period in 3 patients. While all patients had eruption, 10 patients had pruritis, 1 patient had a bullous formation, 1 patient had abdominal pain and 1 patient had attacks of redness throughout the body and a sense of burning in the chest. Two patients had a positive familial history. The diagnosis was confirmed with skin biopsy in all patients. The median follow up time of the patients were 5 years. The patients were treated with H1, H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs. The lesions resolved completely in 4 patients who reached to puberty and 7 patients had marked improvement in a 5.5 year-follow-up period. Ten patients had stabile lesions in a 3.6 year-follow-up period. Conclusions: Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis. The prognosis is good; the disease limits itself and is prone to regress in the adolescent period.

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