Fatih HAŞLAK, Deniz ÖZÇEKER, Zeynep TAMAY

Sütçocuğunda Ender Bir Döküntü Nedeni: Kutanöz Mastositoz

Döküntü ve kaşıntı, sütçocukluğu çağında en sık acil ser- vise başvuru nedenlerinden birisidir. Bu iki semptomun birlikteliğinin birçok hastalığa işaret ediyor olması ayırı- cı tanıyı planlamamızı güçleştirmektedir. Eksiksiz bir öykü ve dikkatli bir fizik muayene bu konuda yardımcıdır. Mastositoz bu döküntü ve kaşıntı ile seyreden hastalıklar arasında ender görülen bir nedendir. Lezyonların alev- lenmelerle seyretmesi, persiste etmesi fizik muayenesinde ise Darier bulgusunun olumlu olması bize bu ender tanıyı düşündürür. Burada mastositoz vakalarına yaklaşımı değerlendirmek amacıyla yakınmalarının başlamasından on altı ay sonra tanı almış ve bu süreçte defalarca palya- tif tedavi almış bir kutanöz mastositoz vakası sunulmuş- tur

Anahtar Kelimeler:

Çocuk, döküntü, mastositoz

A Rare Cause of Eruption in Infancy: Cutaneus Mastocytosis

Skin eruptions and pruritis are one of the most common causes of emergency room admissions. Coexistence of these two symptoms are indicates a wide variety of diseases complicates planning the differential diagnosis. Certainly, a careful and thorough history and physical examination is helpful for the diagnosis. Mastocytosis is a rare condition in the group of disorders characterized by skin eruptions and pruritus. However persisting and exacerbations of eruptions in history and a positive Darier sign in physical examination calls remind this rarely seen diagnosis. In this article, approach to cases with mastocytosis will be evalu- ated by presenting a case with urticeria pigmentosa which was diagnosed sixteen months after patient’s complaints had started who received palliative treatments repeatedly during this period

Keywords:

Child, eruption, mastocytosis,

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1. Arock M, Valent P. Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives. Expert Rev Hematol 2010;3:497-16. http://dx.doi.org/10.1586/ehm.10.42
2. Pardanani A, Akin C, Valent P. Pathogenesis, clinical features, and treatment advances in mastocytosis. Best Pract Res Clin Haematol 2006;19:595-15. http://dx.doi.org/10.1016/j.beha.2005.07.010
3. Valent P, Akin C, Escribano L, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 2007;37:435-53. http://dx.doi.org/10.1111/j.1365-2362.2007.01807.x
4. Escribano L, Akin C, Castells M, et al. Mastocytosis: current concepts in diagnosis and treatment. Ann Hematol 2002; 81:677-90. http://dx.doi.org/10.1007/s00277-002-0575-z
5. Büyüköztürk S. Mastositoz ve anafilaksi; derleme. Turkiye Klinikleri J Immun Allergy-Special Topics 2014;7:53-8.
6. Akoglu G, Erkin G, Çakır B, et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol 2006:20:969-73. http://dx.doi.org/10.1111/j.1468-3083.2006.01696.x
7. Kiszewski AE, Duran-Mckinster C, Orozco-Covarrubias L, Gutierrez-Castrellon P, Ruiz-Maidonaldo R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol 2004;18:285-90. http://dx.doi.org/10.1111/j.1468-3083.2004.00830.x
8. Middelkamp Hup MA, Heide R, Tank B, Mulder PGH, Oranje AP. Comparison of mastocytosis with onset in children and adults. J Eur Acad Dermatol Venereol 2002;16:115- 20. http://dx.doi.org/10.1046/j.1468-3083.2002.00370.x
9. Horny HP, Metcalfe DD, Bennett JM, et al. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL, editors. WHO classi- fication of tumors of hematopoietic and lymphoid tissues. 4th edition. Lyon (France): International Agency for Research and Cancer (IARC), 2008, 54-63.
10. Hartman K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am 2000;14:625-40. http://dx.doi.org/10.1016/S0889-8588(05)70299-9
11. Wimazal F, Geissler P, Shnawa P, Sperr WR,Valent P. Severe life-threatening or disabling anaphylaxis in patients with systemic mastocytosis: a single-center experience. Int Arch Allergy Immunol 2012;157:399-405. http://dx.doi.org/10.1159/000329218
12. Azana JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol 1994;11:102-6. http://dx.doi.org/10.1111/j.1525-1470.1994.tb00560.x
13. Stein DH. Mastocytosis: a review. Pediatr Dermatol 1986; 3:365-75. http://dx.doi.org/10.1111/j.1525-1470.1986.tb00543.x
14. Slavkovic-Jovanovic M, Jovanovic D, Petrovic A, Mihailovic D. Urticaria pigmentosa. A case report. Acta Dermatovenerol APA 2008;7:79-82.
15. Lange M, Niedoszytko M, Renke J, Glén J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol 2013;27:97-102. http://dx.doi.org/10.1111/j.1468-3083.2011.04365.x
16. Rotolo A, Familiari U, Nicoli P, Cilloni D, Saglio G, Guerrasio A. Systemic mastocytosis: an intriguing disorder, hematology-science and practice. In: Lawrie C, ed. Hematology-Science and Practice. In Tech 2012 p.467-85. http://dx.doi.org/10.5772/35471
17. Sahiner MU, Sekerel BE. Urtiker ve anjioodem. Katki Peditari Dergisi 2013;35:139-58.
18. Carter MC, Metcalfe DD. Paediatric mastocytosis. Arch Child 2002;86:315-9. http://dx.doi.org/10.1136/adc.86.5.315
19. Tüysüz ve ark. Mastositoz. Türk Ped Arş 2015;50:108-13.
20. Castells M, Metcalfe DD, Escribano L. Guidelines for the diagnosis and treatment of cutaneous mastocytosis in children. Am J Clin Dermatol 2011;12:259-70. http://dx.doi.org/10.2165/11588890-000000000-00000
21. Heide R, Beishuizen A, De Groot H, Den Hollander JC, Van Doormaal JJ, De Monchy JG, et al. Dutch National Mastocytosis Work Group Mastocytosis in children: a protocol for management. Pediatr Dermatol 2008;25:493-500. http://dx.doi.org/10.1111/j.1525-1470.2008.00738.x
22. Morelli JG. The Skin. In: Behrman RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 19th ed. Philadelphia: WB. Saunders Company; 2011: 2280-82. http://dx.doi.org/10.1016/B978-1-4377-0755-7.00636-9
23. Heide R, de Waard-van der Spek FB, Den Hollander JC, Tank B, Oranje AP. Efficacy of 25% diluted fluticasone propionate 0.05% cream as wet-wrap treatment in cutaneous mastocytosis. Dermatology 2007;214:333-5. http://dx.doi.org/10.1159/000100885