Abstract
Kutanöz mastositoz (KM), dermiste aşırı sayıda mast hücresi (MH)’nin varlığına göre kategorize edilen bir hastalık grubunu tanımlar. KM'li hastalar, sistemik mastositoz için tanı kriterlerini karşılamaz ve dermis dışında herhangi bir organ katılımı kanıtı göstermez. Bu makale tarih, prevalans ve sınıflandırmadan bahsettikten sonra kısaca MH aktivasyonunun farklı klinik özelliklerini ve tetikleyicilerini gözden geçirmekte ve son olarak güncel gözlemler ışığında çocuklarda KM yönetimini ayrıntılı olarak tartışmaktadır. KM'li hastalar, sıklıkla dermal lezyonlar üzerindeki baskılarla başlayan ve MH aracılığıyla meydana gelen semptomlardan muzdariptir. KM yönetimi, esas olarak MH tetikleyicilerinden kaçınma üzerine kuruludur. KM vakalarında tedavinin esasını anafilaksi gibi şiddetli sistemik reaksiyonlarda epinefrin otoenjektörlerinin elde mevcudiyeti ve semptomlar ortaya çıktığında antihistaminiklerin (H1/H2 histamin reseptör blokerleri) kullanılması oluşturmaktadır.
References
- Golkar L, Bernhard DJ. Mastocytosis. Lancet1997; 349: 1379 –1385.
- Sangster A. An anomalous mottled rash, accompanied by pruritus, factitious urticaria and pigmentation, ‘urticaria pigmentosa (?)’ Trans. Clin. Soc. Lond. 1878; 11:161–163.
- Sezary ALCG, Chauvillon P. Dermographisme et mastocytose. Bull. Soc. Fr. Dermatol. Syph. 1936; 43:359–361.
- Sagher FR, Even-Paz Z. Mastocytosis and the Mast Cell. Year Book Medical Publishers, Chicago, 1967.
- Torrelo FA, Navarro CL, Escribano ML, Zambrano AZ. Estudios clínicos y de laboratorio: diagnóstico, tratamiento y clasificación de la mastocitosis pediátrica. Estudio de 172 casos. Actas Dermosif 1998; 89:461-476.
- Kiszewski AE, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol. 2004; 18:285-90.
- Sandru F, Petca RC, Costescu M, et al. Cutaneous mastocytosis in childhood-Update from the Literature. J Clin Med. 2021; 10: 1474.
- Macri A, Cook C. Urticaria Pigmentosa. 2021 Aug 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 29494109.
- Nemat K, Abraham S. Cutaneous mastocytosis in childhood. Allergol Select. 2022; 6:1-10.
- Horny HP, Metcalfe DD, Bennet JM, et al. Mastocytosis. In: WHO Classification of the Tumors of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele H, Vardiman JW. (Eds), IARC, Lyon 2008. p.54
- Meni C, Georgin-Lavialle S, Le Saché de Peufeilhoux L, et al. Paediatric mastocytosis: long-term follow-up of 53 patients with whole sequencing of KIT. A prospective study. Br J Dermatol. 2018; 179: 925–932.
- Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations. Am J Clin Dermatol. 2011; 12:259-70.
- Akoglu G, Erkin G, Cakir B, et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol 2006;20:969–73
- Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J 2005;7:320–2.
- Giona F. Pediatric Mastocytosis: An Update. Mediterr J Hematol Infect Dis. 2021; 13(1): e2021069.
- Lange M, Hartmann K, Carter MC, et al. Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021. Int J Mol Sci. 2021; 22:2586.
- Bonadonna P, Lombardo C, Zanotti R. Mastocytosis and allergic diseases. J Investig Allergol Clin Immunol. 2014; 24:288-97
- Mashiah J, Harel A, Bodemer C, et al. Topical pimecrolimus for pediatric cutaneous mastocytosis. Clin Exp Dermatol. 2018; 43: 559–565.
- Ertugrul A, Baskaya N, Cetin S, Bostanci I. Anaphylaxis and epinephrine autoinjector use in pediatric patients with cutaneous mastocytosis. Pediatr Dermatol. 2021; 38(5):1080-1085. doi: 10.1111/pde.14800.
- Sathishkumar D, Balasundaram A, Mathew SM, et al. Clinicopathological profile of childhood onset cutaneous mastocytosis from a tertiary care center in south India. Indian Dermatol Online J. 2021; 12:706-713.
- Caplan RM. The natural course of urticaria pigmentosa. Analysis and follow-up of 112 cases. Arch Dermatol 1963; 87:146–57.
- Azana JM, Torrelo A, Mediero IG, et al. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatric Dermatology. 1994; 11:102–106.
- Uzzaman A, Maric I, Noel P, Kettelhut BV, Metcalfe DD, Carter MC. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer 2009; 53:629-34.
Abstract
Kutanöz mastositoz (KM), dermiste aşırı sayıda mast hücresi (MH)’nin varlığına göre kategorize edilen bir hastalık grubunu tanımlar. KM'li hastalar, sistemik mastositoz için tanı kriterlerini karşılamaz ve dermis dışında herhangi bir organ katılımı kanıtı göstermez. Bu makale tarih, prevalans ve sınıflandırmadan bahsettikten sonra kısaca MH aktivasyonunun farklı klinik özelliklerini ve tetikleyicilerini gözden geçirmekte ve son olarak güncel gözlemler ışığında çocuklarda KM yönetimini ayrıntılı olarak tartışmaktadır. KM'li hastalar, sıklıkla dermal lezyonlar üzerindeki baskılarla başlayan ve MH aracılığıyla meydana gelen semptomlardan muzdariptir. KM yönetimi, esas olarak MH tetikleyicilerinden kaçınma üzerine kuruludur. KM vakalarında tedavinin esasını anafilaksi gibi şiddetli sistemik reaksiyonlarda epinefrin otoenjektörlerinin elde mevcudiyeti ve semptomlar ortaya çıktığında antihistaminiklerin (H1/H2 histamin reseptör blokerleri) kullanılması oluşturmaktadır.
References
- Golkar L, Bernhard DJ. Mastocytosis. Lancet1997; 349: 1379 –1385.
- Sangster A. An anomalous mottled rash, accompanied by pruritus, factitious urticaria and pigmentation, ‘urticaria pigmentosa (?)’ Trans. Clin. Soc. Lond. 1878; 11:161–163.
- Sezary ALCG, Chauvillon P. Dermographisme et mastocytose. Bull. Soc. Fr. Dermatol. Syph. 1936; 43:359–361.
- Sagher FR, Even-Paz Z. Mastocytosis and the Mast Cell. Year Book Medical Publishers, Chicago, 1967.
- Torrelo FA, Navarro CL, Escribano ML, Zambrano AZ. Estudios clínicos y de laboratorio: diagnóstico, tratamiento y clasificación de la mastocitosis pediátrica. Estudio de 172 casos. Actas Dermosif 1998; 89:461-476.
- Kiszewski AE, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol. 2004; 18:285-90.
- Sandru F, Petca RC, Costescu M, et al. Cutaneous mastocytosis in childhood-Update from the Literature. J Clin Med. 2021; 10: 1474.
- Macri A, Cook C. Urticaria Pigmentosa. 2021 Aug 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 29494109.
- Nemat K, Abraham S. Cutaneous mastocytosis in childhood. Allergol Select. 2022; 6:1-10.
- Horny HP, Metcalfe DD, Bennet JM, et al. Mastocytosis. In: WHO Classification of the Tumors of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele H, Vardiman JW. (Eds), IARC, Lyon 2008. p.54
- Meni C, Georgin-Lavialle S, Le Saché de Peufeilhoux L, et al. Paediatric mastocytosis: long-term follow-up of 53 patients with whole sequencing of KIT. A prospective study. Br J Dermatol. 2018; 179: 925–932.
- Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations. Am J Clin Dermatol. 2011; 12:259-70.
- Akoglu G, Erkin G, Cakir B, et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol 2006;20:969–73
- Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J 2005;7:320–2.
- Giona F. Pediatric Mastocytosis: An Update. Mediterr J Hematol Infect Dis. 2021; 13(1): e2021069.
- Lange M, Hartmann K, Carter MC, et al. Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021. Int J Mol Sci. 2021; 22:2586.
- Bonadonna P, Lombardo C, Zanotti R. Mastocytosis and allergic diseases. J Investig Allergol Clin Immunol. 2014; 24:288-97
- Mashiah J, Harel A, Bodemer C, et al. Topical pimecrolimus for pediatric cutaneous mastocytosis. Clin Exp Dermatol. 2018; 43: 559–565.
- Ertugrul A, Baskaya N, Cetin S, Bostanci I. Anaphylaxis and epinephrine autoinjector use in pediatric patients with cutaneous mastocytosis. Pediatr Dermatol. 2021; 38(5):1080-1085. doi: 10.1111/pde.14800.
- Sathishkumar D, Balasundaram A, Mathew SM, et al. Clinicopathological profile of childhood onset cutaneous mastocytosis from a tertiary care center in south India. Indian Dermatol Online J. 2021; 12:706-713.
- Caplan RM. The natural course of urticaria pigmentosa. Analysis and follow-up of 112 cases. Arch Dermatol 1963; 87:146–57.
- Azana JM, Torrelo A, Mediero IG, et al. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatric Dermatology. 1994; 11:102–106.
- Uzzaman A, Maric I, Noel P, Kettelhut BV, Metcalfe DD, Carter MC. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer 2009; 53:629-34.
Details
| Primary Language | English |
|---|---|
| Subjects | Clinical Sciences |
| Journal Section | Review |
| Authors | |
| Publication Date | February 26, 2024 |
| Submission Date | May 31, 2023 |
| Acceptance Date | January 4, 2024 |
| Published in Issue | Year 2024 Volume: 6 Issue: 1 |
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