Childhood Onset Urticaria Pigmentosa: A Case Report from Indonesian Setting
Abstract
Background: Urticaria pigmentosa (UP) is a rare skin disease caused by increased deposition of clonal mast cell in the skin. C-kit gene mutation is thought to be the cause of UP. The three components of management include therapeutic aspects, counseling, and avoidance of triggering factors that might activate mast cell degranulation. Case report: we reported one case of UP in a 1-year-old boy with a 7-month history of generalized pruritic brownish patches. Dermatological examination revealed generalized hyperpigmented macules, patches and a positive darier's sign. Histopathological examination showed epidermal melanin deposition in the basal layer and mast cell infiltration in the dermal layer which highlighted positively with Giemsa staining. Complete hematology and liver function test were within normal ranges which exclude possibility of internal organ involvement. The initial SCORMA score was 53.4. Patient was treated with a combination of sedative and non-sedative H1-antihistamines with remarkable improvement of the symptoms (SCORMA 39.2). Discussion: Urticaria pigmentosa is rare. Limited definitive data has been obtained regarding the prevalence and clinical profile of this disease. Urticaria pigmentosa most commonly affects children and more than 50% of cases occur before the age of 2 years. The prognosis of UP depends on patient’s age, disease severity, and clinical subtype. In 50-60% of cases will improve during adolescence. Key word: cutaneous mastocytosis, mast cell, darier's signReferences
Sandru F, Petca RC, Costescu M, et al. Cutaneous mastocytosis in childhood—update from the literature. J Clin Med. 2021;10(7):1474.
Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: Practical recommendations. Am J Clin Dermatol. 2011;12(4):259–270.
Solanki PK, Bhide V, Gadage V, Kulkarni V, Patki A. Cutaneous mastocytosis in an infant: Case report and clinicopathological correlation. Glob J Med Clin Case Rep. 2021;8(3):108–111.
Kiszewski AE, Durán-McKinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: A children analysis of 71 cases. J Eur Acad Dermatology Venereol. 2004;18(3):285–290.
Anissa L, Mahri S, Astriningrum R, Agustin T, Rihatmadja R, Rahmayunita G. Bullous Urticaria Pigmentosa in an Infant: A Rare Form of Bullous Disorder. In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), 267–271 (2020). doi:10.5220/0009986302670271
Ben-Amitai D, Metzker A, Cohen H. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J. 2005;7(5):320–322.
Nirmal B, Krishnaram SA, Muthu Y, Rajagopal P. Dermatoscopy of Urticaria Pigmentosa with and without Darier’s Sign in Skin of Colour. Indian Dermatol Online J. 2019;28(10):577–579.
Hartmann K, Escribano L, Grattan C, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; The American Academy of Allergy, Asthma & Immunology; And the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 2016;137(1):35–45.
Heide R, Van Doorn K, Mulder PG, et al. Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis. Clin Exp Dermatol. 2009;34(4):462–468.
Czarny J, Lange M, Ługowska-umer H, Nowicki RJ. Cutaneous mastocytosis treatment : strategies , limitations and perspectives. Postep Dermatol Alergol. 2018;35(6):541–545.
Azaña JM, Torrelo A, Mediero IG, Zambrano A. Urticaria Pigmentosa: A Review of 67 Pediatric Cases. Pediatr Dermatol. 1994;11(2):1
