Turkiye Klinikleri Journal of Pediatrics

.: ORIGINAL RESEARCH
Çocuklarda Kutanöz Mastositoz: Tek Merkez Deneyimi
Pediatric Cutaneous Mastocytosis: Single-Center Experience
Süheyla OCAKa, Esra YÜCELb, Hilal SUSAM ŞENc
aİstanbul Üniversitesi-Cerrahpaşa, Cerrahpaşa Tıp Fakültesi, Çocuk Hematolojisi-Onkolojisi BD, İstanbul, TÜRKİYE
bİstanbul Üniversitesi İstanbul Tıp Fakültesi, Çocuk Alerjisi-İmmünolojisi BD, İstanbul, TÜRKİYE
cAfyonkarahisar Sağlık Bilimleri Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları ABD, Afyonkarahisar, TÜRKİYE
Turkiye Klinikleri J Pediatr. 2020;29(3):148-52
doi: 10.5336/pediatr.2019-73027
Article Language: TR
Full Text
ÖZET
 Amaç: Mastositoz, bir veya daha çok organda mast hücrelerinin birikmesi sonucu oluşan bir hastalıktır. Deri, en sık tutulan organdır. Bu çalışmada, kutanöz mastositozu olan çocuk olguların klinik ve izlem bulgularının tanımlanması amaçlanmıştır. Gereç ve Yöntemler: Ocak 2015-Haziran 2019 tarihleri arasında, 0-18 yaş arasında histopatolojik değerlendirme ile mastositoz tanısı alan 27 olgu çalışmaya dâhil edildi. Olguların yaş, cinsiyet, döküntü süresi, hastalık seyri ve döküntüye ek yakınmaları kaydedildi, ilaç, besin alerjisi, anafilaksi öyküleri sorgulandı. Bulgular: Tüm olgularda, ciltte sınırlı döküntü ile seyreden kutanöz mastositoz görüldü. Makülopapüler kutanöz mastositoz ve soliter mastositoma sırasıyla 24 ve 3 olguda görüldü. Ortanca tanı yaşı 11,5 ay (3 ay-12 yaş) olarak bulundu; 26 olguda döküntü 1 yaş altında ortaya çıkmıştı. Üç olguda, doğum anından itibaren döküntü mevcuttu. Döküntüye ek olarak 11 olguda kaşıntı ve 6 olguda atopik dermatit, 2 olguda astım/reaktif havayolu ve birer olguda tekrarlayan ürtiker ve yüzde sıcak basması atakları öyküsü mevcuttu. Olguların hiçbirinde patolojik boyutta lenfadenopati, splenomegali ya da tam kan sayımında anormal bulgu, eozinofil saptanmadı. Sistemik tutulum olmaması nedeni ile olgular tedavisiz izleme alındı; 6 olguda döküntü sayısında belirgin azalma saptandı ancak hiçbir olguda lezyonlar tamamen gerilemedi. Soliter mastositoma tanılı 3 olgunun lezyonu lokal tedavi ile kayboldu. Atak dönemlerinde lokal ve sistemik tedavi ile tüm olgularda semptom kontrolü sağlandı. Takip süresince hiçbir olguda anafilaksi veya sistemik mastositoza ilerleme görülmedi. Sonuç: Bulgularımız pediatrik mastositozun cilt bulgularıyla sınırlı olduğu ve nadiren sistemik mastositoza dönüştüğü bilgisini desteklemektedir.

Anahtar Kelimeler: Mastositoz; çocukluk çağı; kutanöz
ABSTRACT
 Objective: Mastocytosis is a disease characterized by the accumulation of mast cells in one or more organ systems. The skin is the most commonly affected organ. In this study, it is aimed to present the clinical findings and follow-up of pediatric patients with cutaneous mastocytosis. Material and Methods: Between January 2015 and June 2019, 27 cases between the ages of 0-18 who were diagnosed as mastocytosis by histopathological evaluation were included in the study. Age, gender, duration of rash, disease course and additional complaints were recorded, drug, food allergy, and anaphylaxis were questioned. Results: Cutaneous mastocytosis with limited skin rash was observed in all cases. Maculopapular cutaneous mastocytosis and solitary mastocytoma were seen in 24 and 3 cases, respectively. The median age at diagnosis was 11,5 months (3 months-12 years); the rash occurred in 26 patients under 1 year of age. Three patients had a rash from birth. In addition to the rash, there were pruritus in 11 cases, atopic dermatitis in 6 cases, asthma/reactive airway in 2 cases, recurrent urticaria and flashing attacks in 1 case each. None of the patients had pathological lymphadenopathy, splenomegaly or abnormal findings on complete blood count, and eosinophilia. The cases were monitored without treatment due to the absence of systemic involvement; there was a significant decrease in the number of rashes in 6 cases, but none of the lesions completely regressed in the follow-up period. Lesions of 3 cases with solitary mastocytoma disappeared with local treatment. Symptom control was achieved in all cases with local and systemic treatment during episodes. Anaphylaxis or progression of systemic mastocytosis wasn't observed during the follow-up period. Conclusion: Our findings support the knowledge that pediatric mastocytosis is limited to skin findings and rarely progresses to systemic mastocytosis.

Keywords: Mastocytosis; childhood; cutaneous
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