A Cutaneous mastocytosis: A self-limiting benign disease in a series of 45 cases
DOI:
https://doi.org/10.66344/jpad.v32i2.1910Abstract
Background Cutaneous mastocytosis is a rare disease. It results from a hyperplastic response of mast cells to abnormal stimuli due of activating c-KIT mutations.
Objective To collect all cases of cutaneous mastocytosis and evaluate them clinically and histopathologically.
Methods This is a case series descriptive study where cutaneous mastocytosis cases were collected during the period from 2017-2021. Full history and clinical evaluation were carried out. Skin biopsies were processed and stained with Giemsa stain and CD117 IHC marker to evaluate histopathological changes. Therapeutic trial using antihistamine and corticosteroids were conducted.
Results Analysis of forty five cases with the classical cutaneous mastocytosis was carried out involving 26 males (57.77%) and 19 females (42.22%). Their ages ranged from 1-15 years with a mean±SD of 3.5±3.7 years while the age of onset was mostly after the age of one year. All patients had itching with variable severity. Forty-two (93.3%) patients had a generalized rash while 3(6.7%) cases had localized lesions. The rash was dark brown maculopapular rash in 35 (77.77%), bullous in 4(8.88%), plaque in 3 (6.66%), nodular in 2 (4.44%), and xanthogranuloma like in 1(2.22%) case. The disease was slow and regressing except bullous variant which ran an aggressive course. Histopathology showed mast cells proliferation in the superficial and deep dermis and sub-epidermal blisters in four patients. All patients fulfilled the diagnostic criteria of cutaneous mastocytosis. All patients responded to therapy and their rash and symptoms were relieved.
Conclusion Cutaneous mastocytosis is a disease of children that runs a benign course and then regresses spontaneously. They all needed symptomatic treatment until they had a full remission.
References
1. Sánchez-Borges M., Asero R., Ansotegui I.J., Baiardini I., Bernstein J.A., Canonica G.W., Gower R., Kahn D.A., Kaplan A.P., Katelaris C., et al. Diagnosis and treatment of urticaria and angioedema: A worldwide perspective. World Allergy Organ J. 2012;5:125-147.
2. Nettleship J, Tay W. Rare forms of urticaria. Br Med J. 1869;2:323-4.
3. Sangster A. An anomalous mottled rash, accompanied by pruritus, factitious urticaria and pigmentation, ‘urticariapigmentosa. Trans Clin Soc Lond. 1878;11:161-3.
4. Valent P. Diagnostic evaluation and classification of mastocytosis. Immunol. Allergy Clin N Am. 2006;26:513-34.
5. Patnaik M.M., Rindos M., Kouides P.A., Tefferi A., Pardanani A. Systemic mastocytosis: A concise clinical and laboratory review. Arch Pathol Lab Med. 2007;131:784-91.
6. Pardanani A. Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management. Am J Hematol. 2018;94:363-77.
7. Castells M.C., Akin C. Mastocytosis (cutaneous and systemic): Evaluation and diagnosis in children. [(accessed on 5 December 2020)]; Up to date. 2018 Available online: https://www.uptodate.com/contents/mastocytosis-cutaneous-and-systemic-evaluation-and-diagnosis-in-children.
8. Torrelo A., Alvarez-Twose I., Escribano L. Childhood mastocytosis. Curr Opin Pediatr. 2012;24:480-6.
9. Ertugrul A., Bostanci I., OzturkKaymak A., Gurkan A., Ozmen S. Pediatric cutaneous mastocytosis and c-KIT mutation screening. Allergy Asthma Proc. 2019;40:123-8.
10. Leung A.K.C., Lam J.M., Leong K.F. Childhood solitary cutaneous mastocytoma: Clinical manifestations, diagnosis, evaluation, and management. Curr Pediatr Rev. 2019;15:42-6.
11. Ferrante G, Scavone V, Muscia MC, Adrignola E, Corsello G, Passalacqua G, La Grutta S. The care pathway for children with urticaria, angioedema, mastocytosis. World Allergy Organ J. 2015;8:5-10.
12. Slavković-Jovanović M., Jovanović D., Petrović A., Mihailović D. Urticariapigmentosa. A case report. Acta Dermatovenerol Alp Pannon Adriat. 2008;17:79-82.
13. Azaña J.M., Torrelo A., Mediero I.G., Zambrano A. Urticariapigmentosa: A review of 67 pediatric cases. Pediatr Dermatol. 1994;11:102-6.
14. Murphy M., Walsh D., Drumm B., Watson R. Bullous mastocytosis: A fatal outcome. Pediatr Dermatol. 1999;16:452-5.
15. Cengizlier R., Hücümenoğlu S., Özen A., TülinŞayli R. Treatment of telangiectasia macularis eruptiva perstans with montelukast. Allergol Immunopathol. 2009;37:334-6.
16. Bergström A, Rollman O, Emtestam L, Mattson M, Hägglund H, Nilsson G, et al. [Cutaneous mastocytosis-update and clinical guidelines]. Lakartidningen. 2018;115:FASY.
17. Méni C, Bruneau J, Georgin-Lavialle S, Le Saché de Peufeilhoux L Damaj G, Hadj-Rabia S, et al. Paediatricmastocytosis: a systematic review of 1747 cases. Br J Dermatol. 2015;172(3):642-51.
18. Hartmann K., Metcalfe D.D. Pediatric mastocytosis. Hematol Clin N Am. 2000;14:625-40.
19. Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations. Am J Clin Dermatol. 2011;12(4):259-70.
20. Lange M, Nedoszytko B, Górska A, Zawrocki A, Sobjanek M, Kozłowski D. Mastocytosis in children and adults: Clinical disease heterogeneity. Arch Med Sci. 2012;8(3):533-41.
21. Al- Hamami HR, Al-Mashaddani SA, Fadheel BM, Al-Itabi AA Medicine C. Chronic Ordinary Urticaria : A Clinico- Epidemiological Study in Iraqi Patients. IJSR. 2016;5(6):10-3.
22. Singalavanija S, Limpongsanurak W. Cutaneous mastocytosis in Thai children. J Med Assoc Thai. 2008 ;91 Suppl 3:S143-6.
23. Gürkan A, Arda N, Tekgunduz S, Toprak Ş, Ozmen S. Cutaneous Mastocytosis in Children: Retrospective Analysis of 41 Patients. Turkiye Klin J Dermatology. 2016;21;26:10-4.
24. Lange M , Zawadzka A , Schrörs S , Słomka J , Hanna Umer HL Nedoszytko B et al. The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: A single-center experience. Postepy Dermatol Alergol. 2017;34(4):306-12.
25. Cohen P. Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment. Dermatol Pract Concept. 2016;6(3):31-8.
26. Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 17326 children. Australas J Dermatol. 2001;42(1):15-21.
27. Nabavi NS, Nejad MH, Feli S, Bakhshoodeh B, Layegh P. Adult Onset of XanthelasmoidMastocytosis: Report of a Rare Entity. Indian J Dermatol. 2016;61(4): 468.
28. Alotaibi L, Alali F, Abdullgaffar B, Keloth T, Alhammadi A. Combined central xanthomatous and peripheral macular skin lesions in an infant. Int J Dermatol. 2014;53(6):673-5.
29. Srivastava N, Chand S, Singh S. Pseudoxanthomatous mastocytosis. Int J Dermatol. 2008;47(1):50-1.
30. Huang A, Fiadorchanka N,Brar K, Balderacchi JL, Glick SA. In utero presentation of aggressive systemic mastocytosis in a neonate. Br J Dermatol. 2017;177:1439-41.
31. Macias, V. C., Amaro, C., Freitas, I., Vieira, R., & Cardoso, J. Cutaneous Mastocytosis In Childhood - Retrospective study of 32 patients. J Portuguese Soc Dermatol Venereol. 2012;70(4):459-63.
32. Sinha P, Sandhu S, Sen A, Sood A. Pseudoxanthomatous or xanthelasmoid mastocytosis: Reporting a rare entity. Indian J Dermatol Venereol Leprol. 2020;86:382-5.
33. Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008;63(2):226-32.
34. Al-Ghamdi AH, Al-Ghamdi HS, AlSohaimi AAA, Al-Ghamdi M. Urticaria pigmentosa in a child with sporadic bilateral diffuse conjunctival redness: Case report and review of the literature. J Pak Assoc Dermatol. 2017;27(2):192-6.
35. Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129(11):1420-8.
36. Czarny J, Lange M, Ługowska-Umer H, Nowicki RJ. Cutaneous mastocytosis treatment: strategies, limitations and perspectives. Postep Dermatologii I Alergol. 2018;35(6):541-5.
37. Munshi N, Varma K, Kumar UK. Urticaria pigmentosa: A rare case report. IP Indian J Clin Exp Dermatol. 2018;4(4):348-9.
38. Frieri M, Quershi M. Pediatric mastocytosis: A review of the literature. Pediatr Allergy Immunol Pulmonol. 2013;26(4):175-80.
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