Abstract
Abstract
Mastocytosis is a disease group characterized by skin lesions and/or systemic involvement developing as a result of mast cell proliferation. The etiology is unknown. Ledby the skin it may involve bone marrow, bones, gastrointestinal system, liver, spleen andlymph glands. It is divided into two groups as cutaneous and systemic. Cutaneous mastocytosis are observed in childhood with the main types being urticaria pigmentosa, diffuse cutaneous mastocytosis and isolated mastocytoma. In mastocytosis, patients maybe observed to have itching, flushing, urticaria, abdominal pain, diarrhea and cardiovascular symptoms due to release of mast cell mediators. Urticaria pigmentosa (UP) is themost commonly seen cutaneous mastocytosis. Lesions are pink, red or brown in colorin the form of macules, papules and nodules. Generally, it occurs in the first six monthsof life and enters remission after puberty. Here we present the clinical and histopatho-logical findings of a six-month old male infant with UP diagnosis.
Keywords
References
- Kaynaklar 1.Suh JH, Park KY, Seo SJ. Urticaria Pigmentosa MimickingMultiple Lentigine-like Brownish Macules in a 22-Month-OldBoy. J Korean Med Sci. 2017 Jun;32(6):885-886. 2.Güzeloğlu M, Özkaya E, Metin A, Uğraş S, Cesur Y, OdabaşD. Urtikerya Pigmentoza: Bir Olgu Sunumu.T Klin Pediat-ri:1998;7:96-99 3.Ghiasi M, Ghanadan A, Jesri SB, Sotudeh S, Ramyar A. Dif-fuse cutaneous mastocytosis: report of a severe case with fa-tal outcome. Dermatol Online J. 2011 Mar 15;17(3):7. 4.Guevara BEK, Guillano VP, Dayrit JF. Urticaria pigmento-sa with concomitant polycythaemia vera in a 3-year-old boy.Clin Exp Dermatol. 2017 Aug;42(6):696-698. 5. Ocampo-Candiani J, García-García SC, Guerrero-GonzálezGA, Martínez-Cabriales SA. Disseminated brown macules inan infant. Aust Fam Physician. 2015 Sep;44(9):657-9. 6.Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shos-han M. Natural history and treatment of cutaneous and syste-mic mastocytosis. Postgrad Med. 2017 Nov;129(8):896-901
Abstract
Öz
Mastositoz, mast hücrelerinin proliferasyonu sonucu gelişen deri lezyonları ve/veya sistemik tutulum ile karakterize olan bir hastalık grubudur. Etyolojisi bilinmemektedir. Deri başta olmak üzere, kemik iliği, kemikler, gastrointestinal sistem, karaciğer, dalak ve lenf bezleri tutabilir. Kutanöz ve sistemik olmak üzere iki gruba ayrılır. Kutanöz mastositozların çocuklukta görülen başlıca tipleri ürtikerya pigmentoza, diffüz kutanöz mastositoz ve izole mastositomdur. Mastositozlarda mast hücre mediyatörlerin salınması ile hastalarda kaşıntı, flushing, ürtiker, abdominal ağrı, diare, kardiyovasküler semptomlargörülebilir. Ürtikerya Pigmentosa (ÜP) en sık görülen kutanöz mastositozdur. Lezyonlar pembe, kırmızı ve kahverengi renklerde makül, papül ve nodüller şeklinde görülebilir. Genellikle yaşamın ilk altı ayında ortaya çıkar ve puberteden sonra remisyona girer. Burada klinik ve histopatolojik olarak ÜP tanısı konulan altı aylık erkek bebek olgusu sunulmuştur.
Keywords
References
- Kaynaklar 1.Suh JH, Park KY, Seo SJ. Urticaria Pigmentosa MimickingMultiple Lentigine-like Brownish Macules in a 22-Month-OldBoy. J Korean Med Sci. 2017 Jun;32(6):885-886. 2.Güzeloğlu M, Özkaya E, Metin A, Uğraş S, Cesur Y, OdabaşD. Urtikerya Pigmentoza: Bir Olgu Sunumu.T Klin Pediat-ri:1998;7:96-99 3.Ghiasi M, Ghanadan A, Jesri SB, Sotudeh S, Ramyar A. Dif-fuse cutaneous mastocytosis: report of a severe case with fa-tal outcome. Dermatol Online J. 2011 Mar 15;17(3):7. 4.Guevara BEK, Guillano VP, Dayrit JF. Urticaria pigmento-sa with concomitant polycythaemia vera in a 3-year-old boy.Clin Exp Dermatol. 2017 Aug;42(6):696-698. 5. Ocampo-Candiani J, García-García SC, Guerrero-GonzálezGA, Martínez-Cabriales SA. Disseminated brown macules inan infant. Aust Fam Physician. 2015 Sep;44(9):657-9. 6.Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shos-han M. Natural history and treatment of cutaneous and syste-mic mastocytosis. Postgrad Med. 2017 Nov;129(8):896-901
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | makaleler |
| Authors | |
| Publication Date | January 30, 2019 |
| Published in Issue | Year 2019 Volume: 11 Issue: 1 |
