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

Yıl 2021, Cilt 21, Sayı 2, 198 - 202, 20.09.2021

Öz

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.

Kaynakça

  • 1. Valent P, Horny H-P, Escribano L, LongleyB J, LiC Y, SchwartzL B, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leukemia Research 2001;25:603-25. google scholar
  • 2. Hartman K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am 2000;14:625-40. google scholar
  • 3. Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O, et al. Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future. Cancer Res 2017; 77(6):1261-70. google scholar
  • 4. Ferrante G, Scavone V, Muscia MC, Adrignola M, Corsello G, Passalacqua G, et al. The care pathway for children with urticaria, angioedema, mastocytosis. World Allergy Organ J 2015;8:5. google scholar
  • 5. Azana JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol 1994;11:102-6. google scholar
  • 6. Carter MC, Metcalfe DD. Paediatric mastocytosis. Arch Child 2002;86:315-9. google scholar
  • 7. Zeynep Tamay, Deniz Özçeker Current approach to cutaneous mastocytosis in childhood Turk Pediatri Ars 2016;51:123-7. google scholar
  • 8. KiszewskiAE, Duran-Mckinster C, Covarrubias LO, Castrellon PG, Maldonado RR. Cutaneous mastocytosis in children: a clinical analysis of 71 cases AE 2004 European Academy of Dermatology and Venereology JEADV 2004;18:285-90. google scholar
  • 9. Serarslan G, Atik E, Canda Ş. Pediatric Cutaneous Mastocytosis: Demographic, Clinical and Histopathological Findings. Turkish Journal of Dermatology 2008;2:69-72. google scholar
  • 10. 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. google scholar
  • 11. Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J 2005;7:320-2. google scholar
  • 12. G Akoglu, G Erkin, B Cakir, G Boztepe, S Sahin, A Karaduman, et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. European Academy of Dermatology and Venereology 2006;20:969-73. google scholar
  • 13. Meni C, Bruneau J, Georgin-Lavialle S, Peufeilhoux LS, Damaj G, Hadj-Rabia S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 2015;172(3):642-51. google scholar
  • 14. Heide R, Tank B, Oranje AP. Mastocytosis in childhood. Pediatr Dermatol 2002;19:375-81. google scholar
  • 15. Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol 2001;42:15-21. google scholar
  • 16. Tüysüz G, Özdemir N, Apak H, Kutlubay Z, Demirkesen C, Celkan T. Childhood mastocytosis: results of a single center Türk Ped Arş 2015;50:108-13. google scholar
  • 17. Alvarez-Twose I, Vano-Galvan S, Sanchez-Munoz L, Morgado JM, Matito A, Torrelo A, et al. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012;-67:813-21. google scholar
  • 18. Kettlehut B, Metcalfe D. Pediatric mastocytosis. J Invest Dermatol 1991;96:15-8. google scholar

Çocuklarda Kutanöz Mastositoz: Sadece Bir Deri Hastalığı mı?

Yıl 2021, Cilt 21, Sayı 2, 198 - 202, 20.09.2021

Öz

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.

Kaynakça

  • 1. Valent P, Horny H-P, Escribano L, LongleyB J, LiC Y, SchwartzL B, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leukemia Research 2001;25:603-25. google scholar
  • 2. Hartman K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am 2000;14:625-40. google scholar
  • 3. Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O, et al. Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future. Cancer Res 2017; 77(6):1261-70. google scholar
  • 4. Ferrante G, Scavone V, Muscia MC, Adrignola M, Corsello G, Passalacqua G, et al. The care pathway for children with urticaria, angioedema, mastocytosis. World Allergy Organ J 2015;8:5. google scholar
  • 5. Azana JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol 1994;11:102-6. google scholar
  • 6. Carter MC, Metcalfe DD. Paediatric mastocytosis. Arch Child 2002;86:315-9. google scholar
  • 7. Zeynep Tamay, Deniz Özçeker Current approach to cutaneous mastocytosis in childhood Turk Pediatri Ars 2016;51:123-7. google scholar
  • 8. KiszewskiAE, Duran-Mckinster C, Covarrubias LO, Castrellon PG, Maldonado RR. Cutaneous mastocytosis in children: a clinical analysis of 71 cases AE 2004 European Academy of Dermatology and Venereology JEADV 2004;18:285-90. google scholar
  • 9. Serarslan G, Atik E, Canda Ş. Pediatric Cutaneous Mastocytosis: Demographic, Clinical and Histopathological Findings. Turkish Journal of Dermatology 2008;2:69-72. google scholar
  • 10. 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. google scholar
  • 11. Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J 2005;7:320-2. google scholar
  • 12. G Akoglu, G Erkin, B Cakir, G Boztepe, S Sahin, A Karaduman, et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. European Academy of Dermatology and Venereology 2006;20:969-73. google scholar
  • 13. Meni C, Bruneau J, Georgin-Lavialle S, Peufeilhoux LS, Damaj G, Hadj-Rabia S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 2015;172(3):642-51. google scholar
  • 14. Heide R, Tank B, Oranje AP. Mastocytosis in childhood. Pediatr Dermatol 2002;19:375-81. google scholar
  • 15. Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol 2001;42:15-21. google scholar
  • 16. Tüysüz G, Özdemir N, Apak H, Kutlubay Z, Demirkesen C, Celkan T. Childhood mastocytosis: results of a single center Türk Ped Arş 2015;50:108-13. google scholar
  • 17. Alvarez-Twose I, Vano-Galvan S, Sanchez-Munoz L, Morgado JM, Matito A, Torrelo A, et al. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012;-67:813-21. google scholar
  • 18. Kettlehut B, Metcalfe D. Pediatric mastocytosis. J Invest Dermatol 1991;96:15-8. google scholar

Ayrıntılar

Birincil Dil İngilizce
Konular Pediatri
Bölüm Araştırma Makaleleri
Yazarlar

Tuğba AKTÜRK (Sorumlu Yazar)
Hakkari State Hospital, Department of Pediatrics, Istanbul, Turkey
0000-0002-7136-3023
Türkiye


Emine TÜRKKAN
University of Health Sciences, Prof. Dr. Cemil Taşcıoglu City Hospital, Department of Pediatric Hematology, Istanbul, Turkey
0000-0002-5126-7843
Türkiye


Deniz ÖZÇEKER
University of Health Sciences, Prof. Dr. Cemil Taşcıoglu City Hospital, Department of Pediatric Allergy and Immunolgy, Istanbul, Turkey
0000-0002-0032-6727
Türkiye

Yayımlanma Tarihi 20 Eylül 2021
Yayınlandığı Sayı Yıl 2021, Cilt 21, Sayı 2

Kaynak Göster

Bibtex @araştırma makalesi { jchild896136, journal = {Çocuk Dergisi}, issn = {}, eissn = {1308-8491}, address = {Çocuk Dergisi Yayın Kurulu Sekreterliği İstanbul Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çapa, 34390, İstanbul}, publisher = {İstanbul Üniversitesi}, year = {2021}, volume = {21}, pages = {198 - 202}, doi = {}, title = {Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?}, key = {cite}, author = {Aktürk, Tuğba and Türkkan, Emine and Özçeker, Deniz} }
APA Aktürk, T. , Türkkan, E. & Özçeker, D. (2021). Cutaneous Mastocytosis in Children: Is It Just a Skin Disease? . Çocuk Dergisi , 21 (2) , 198-202 . Retrieved from https://dergipark.org.tr/tr/pub/jchild/issue/64961/896136
MLA Aktürk, T. , Türkkan, E. , Özçeker, D. "Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?" . Çocuk Dergisi 21 (2021 ): 198-202 <https://dergipark.org.tr/tr/pub/jchild/issue/64961/896136>
Chicago Aktürk, T. , Türkkan, E. , Özçeker, D. "Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?". Çocuk Dergisi 21 (2021 ): 198-202
RIS TY - JOUR T1 - Cutaneous Mastocytosis in Children: Is It Just a Skin Disease? AU - Tuğba Aktürk , Emine Türkkan , Deniz Özçeker Y1 - 2021 PY - 2021 N1 - DO - T2 - Çocuk Dergisi JF - Journal JO - JOR SP - 198 EP - 202 VL - 21 IS - 2 SN - -1308-8491 M3 - UR - Y2 - 2021 ER -
EndNote %0 Çocuk Dergisi Cutaneous Mastocytosis in Children: Is It Just a Skin Disease? %A Tuğba Aktürk , Emine Türkkan , Deniz Özçeker %T Cutaneous Mastocytosis in Children: Is It Just a Skin Disease? %D 2021 %J Çocuk Dergisi %P -1308-8491 %V 21 %N 2 %R %U
ISNAD Aktürk, Tuğba , Türkkan, Emine , Özçeker, Deniz . "Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?". Çocuk Dergisi 21 / 2 (Eylül 2021): 198-202 .
AMA Aktürk T. , Türkkan E. , Özçeker D. Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?. Çocuk Dergisi. 2021; 21(2): 198-202.
Vancouver Aktürk T. , Türkkan E. , Özçeker D. Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?. Çocuk Dergisi. 2021; 21(2): 198-202.
IEEE T. Aktürk , E. Türkkan ve D. Özçeker , "Cutaneous Mastocytosis in Children: Is It Just a Skin Disease?", Çocuk Dergisi, c. 21, sayı. 2, ss. 198-202, Eyl. 2021