Ayşe Serap KARADAĞ, Serap BİLGİLİ GÜNEŞ, Zennure TAKCI, Mustafa KÖSEM, Ömer ÇALKA

Comparison of cutaneous mastocytosis with onset in children and adults*

Background/aim: Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and sometimes other organs. This study aimed to compare the demographic, clinical, and histopathological findings of cutaneous mastocytosis with onset in children and in adults. Materials and methods: Patients diagnosed with cutaneous mastocytosis in 2 different dermatology clinics between 2007 and 2011 were included in the study. Demographic characteristics of the patients as well as localization and type of the cutaneous lesions, presence of symptoms, Darier s sign, family history, systemic involvement, and histopathological evaluations were retrospectively examined. Results: Out of the 30 cases of cutaneous mastocytosis, 60% of patients were male (n = 18) and 40% were female (n = 12). Twenty-two patients had childhood-onset mastocytosis (≤15 years) and 8 patients had adult-onset mastocytosis. The onset of the disease occurred before the age of 2 years in all cases of childhood onset. Patients with adult-onset mastocytosis had statistically significantly more systemic involvement than those with childhood-onset mastocytosis (P < 0.05). Conclusion: Cutaneous mastocytosis is a benign disease in children without systemic involvement and is usually sporadic.

PDF

___

1. Briley LD, Phillips CM. Cutaneous mastocytosis: a review focusing on the pediatric population. Clin Pediatr (Phila) 2008; 47: 757761.
2. Heide R, Beishuizen A, De Groot H, Den Hollander JC, Van Doormaal JJ, De Monchy JG, Pasmans SG, Van Gysel D, Oranje AP; Dutch National Mastocytosis Work Group. Mastocytosis in children: a protocol for management. Pediatr Dermatol 2008; 25: 493500.
3. Kutlubay Z, Yardımcı G, Engin B, Tüzün Y. Cutaneous mastocytosis. J Turk Acad Dermatol 2011; 5: 1153r1.
4. Amon U, Hartmann K, Horny HP, Nowak A. Mastocytosis an update. J Dtsch Dermatol Ges 2010; 8: 695711.
5. Hup MA, Heide R, Tank B, Mulder PG, Oranje AP. Comparison of mastocytosis with onset in children and adults. J Eur Acad Dermatol Venereol 2002; 16: 115120.
6. 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: 285290.
7. Lange M, Niedoszytko M, Renke J, Gleń J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol 2013; 27: 97102.
8. Serarslan G, Atik E, Canda Ş. Pediatric cutaneous mastocytosis: demographic, clinical and histopathological findings. Turk J Dermatol 2008; 2: 6972 (article in Turkish with an abstract in English).
9. Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J 2005; 7: 320322.
10. Akoglu G, Erkin G, Cakir B, Boztepe G, Sahin S, Karaduman A, Atakan N, Akan T, Kolemen F. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol 2006; 20: 969973.
11. Kanwar AJ, Sandhu K. Cutaneous mastocytosis in children: an Indian experience. Pediatr Dermatol 2005; 22: 8789.
12. Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Aust J Dermatol 2001; 42: 1521.
13. Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res 2001; 25: 519 528.
14. Calka O. Urticaria pigmentosa. In: Tüzün Y, Gürer MA, Serdaroğlu S, Oğuz O, Aksungur VL, editors. Dermatoloji. 3rd ed. İstanbul, Turkey: Nobel Tıp Kitabevleri; 2008. pp. 2005 2010 (in Turkish).
15. Rosbotham JL, Malik NM, Syrris P, Jeffery S, Bedlow A, Gharraie S, Murday VA, Holden CA, Carter ND. Lack of c-kit mutation in familial urticaria pigmentosa. Br J Dermatol 1999; 140: 849852.
16. Fowler JF Jr, Parsley WM, Cotter PG. Familial urticaria pigmentosa. Arch Dermatol 1986; 122: 8081.
17. Oku T, Hashizurne H, Yokote R, Sano T, Yamada M. The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). Arch Dermatol 1990; 126: 14781484.
18. Anstey A, Lowe DG, Kirby JD, Horton MA. Familial mastocytosis: a clinical, immunophenotypic, light and electron microscopic study. Br J Dermatol 1991; 125: 583587.
19. Prignano F, Troiano M, Lotti T. Cutaneous mastocytosis: successful treatment with narrowband ultraviolet B phototherapy. Clin Exp Dermatol 2010; 35: 914915.
20. Lange M, Nedoszytko B, Górska A, Zawrocki A, Sobjanek M, Kozlowski D. Mastocytosis in children and adults: clinical disease heterogeneity. Arch Med Sci 2012; 8: 533541.
21. Ben-Amitai D, Metzter A. Pediatric mastocytosis. Ann Dermatol Venereol 1998; 125: 196.
22. Lanternier F, Cohen-Akenine A, Palmerini F, Feger F, Yang Y, Zermati Y, Barète S, Sans B, Baude C, Ghez D et al. Phenotypic and genotypic characteristics of mastocytosis according to the age of onset. PLoS One 2008; 3: e1906.
23. Valent P. Diagnostic evaluation and classification of mastocytosis. Immunol Allergy Clin North Am 2006; 26: 515 534.
24. Pardanani A, Akin C, Valent P. Pathogenesis, clinical features, and treatment advances in mastocytosis. Best Pract Res Clin Haematol 2006; 19: 595615.