Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?

Abstract

Introduction: Mastocytosis is characterized by the excessive proliferation and accumulation of mast cells in organs such as the spleen, liver, lymph nodes, skin, gastrointestinal tract, and bone marrow. Cutaneous mastocytosis, which is only presented with skin involvement, is frequently seen in childhood. Objective: It was aimed to determine the demographic and clinical characteristics of patients with cutaneous mastocytosis and the knowledge level of families about the disease, and to raise awareness about this disease. Material and Methods: The records of 25 cases with cutaneous mastocytosis were evaluated retrospectively and the knowledge levels of the families were measured with a questionnaire. Results: The mean follow-up period of 25 patients was 39 months. The most common type of the disease was urticaria pigmentosa (60%). Itching (n=14) was the most common symptom in patients. Only 2 patients were carrying adrenaline auto-injectors. While 60% of the families (n=15) had fears about the disease; of these 15 families, 53.3% (n=8) were afraid of the re-spread of the disease, 13.3% (n=2) turning of the disease into skin cancer, 13.3% (n=2) occupation of the internal organs, 13.3% (n=2) having a new attack, and 0.4% (n=1) re-forming of the spots. Conclusion: The cutaneous forms of mastocytosis are mostly observed in childhood, and systemic involvement is rare. Mastocytosis progresses with rashes in childhood and its findings are limited to the skin. Families should be better informed about mastocytosis and the need for patients to carry an adrenaline auto-injector due to the risk of attacks should be explained to them.

Keywords

• Anaphylaxis
• cutaneous mastocytosis
• mastocytosis

Çocuklarda Kutanöz Mastositoz: Sadece Bir Deri Hastalığı mı?

Abstract

Giriş: Mastositoz, mast hücrelerinin dalak, karaciğer, lenf nodu, deri, gastrointestinal yol, kemik iliği gibi organlarda aşırı çoğalması ve birikmesi ile karakterize bir hastalıktır. Sıklıkla çocukluk çağında sadece deri tutulumu ile seyreden kutanöz mastositoz tipi görülür. Amaç: Kutanöz mastositozlu hastaların demografik ve klinik özelliklerini belirlemek, ailelerin hastalık hakkında bilgi düzeylerini ölçmek ve bu hastalık hakkında farkındalık oluşturmak amaçlanmıştır. Gereç ve Yöntemler: Kutanöz mastositoz tanısı alan 25 olgunun dosyası geriye dönük olarak değerlendirildi ve ailelere kutanöz mastositoz ile ilgili bilgi düzeylerini ölçmek için anket uygulandı. Bulgular: İzlem süresi ortalama 39 ay olan 25 kutanöz mastositozlu hasta çalışmaya dahil edildi. Hastaların %60’ı ürtikerya pigmenzota tipinde idi. Kaşıntı en sık olarak görülen semptom (n=14) idi. Sadece 2 hastamız adrenalin oto enjektörü taşımaktaydı. Ailelerin %60’ında (n=15) hastalık ile ilgili korkular mevcuttu; bu 15 ailenin %53,3’ü (n=8) hastalığın tekrar yayılmasından, %13,3’ü (n=2) cilt kanserine dönüşmesinden, %13,3’ü (n=2) iç organların tutulmasından, %13,3’ü (n=2) atak geçirmesinden, %0,4’ü (n=1) lekelerin tekrar oluşmasından korkmakta idi Sonuç: Mastositoz çocukluk çağında döküntülerle beraber seyreden ve kendini cilt bulguları ile sınırlayan bir hastalıktır. Çocukluk çağında daha çok kutanöz formu görülen hastalığın sistemik tutulumu nadir görülür. Ailelerin mastositoz hastalığı hakkında bilgilendirilmesi ve atak geçirme riskine yönelik adrenalin oto enjektörü taşıması gerekliliği anlatılmalıdır.

Keywords

• Anafilaksi
• kutanöz mastositoz
• mastositoz

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