Bullous Urticaria Pigmentosa in an Infant: A Rare Form of Bullous Disorder

Lidwina Anissa, Sarah Mahri, Rinadewi Astriningrum, Triana Agustin, Rahadi Rihatmadja, Githa Rahmayunita

2019

Abstract

Mastocytosis is a rare, sporadic, and a heterogeneous group of hematopoietic disorder, characterized by an enormous number and accumulation of mast cells in one or more organ systems. The prevalence of mastocytosis is challenging to determine due to underdiagnosis. Pediatric-onset mastocytosis which was commonly diagnosed before two years of age is generally a benign disease. The course of pediatric-onset mastocytosis is variable, from birth to the first year of life, with an average of 2.5 months. Cutaneous mastocytosis may manifest as urticaria pigmentosa, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptive perstans. Bullous urticaria pigmentosa is a rare variant of urticaria pigmentosa. Blistering is considered to be an effect of free mediator activity. The symptoms are mostly in proportion to the mast cell degranulating activities in tissues, which may appear in the first year of life. Although systemic involvement is rare in pediatric cutaneous mastocytosis, blistering may promote secondary infection and electrolyte imbalance. We report a four-month-old infant with bullous urticaria pigmentosa. The symptoms had appeared since the second day of life. Routine hematology examination revealed mild microcytic hypochromic anemia. Skin biopsy from the lesional skin revealed diffuse dermal infiltration of mast cells, some showing granules and scanty cytoplasm which supported the diagnosis of urticaria pigmentosa. The patient was managed with antihistamines. In two-month-period of follow up, the development of new lesions is slowing.

Paper Citation

in Harvard Style

Anissa L., Mahri S., Astriningrum R., Agustin T., Rihatmadja R. and Rahmayunita G. (2019). 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 - Volume 1: ICTROMI, ISBN 978-989-758-469-5, pages 267-271. DOI: 10.5220/0009986302670271

in Bibtex Style

@conference{ictromi19,
author={Lidwina Anissa and Sarah Mahri and Rinadewi Astriningrum and Triana Agustin and Rahadi Rihatmadja and Githa Rahmayunita},
title={Bullous Urticaria Pigmentosa in an Infant: A Rare Form of Bullous Disorder},
booktitle={Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease - Volume 1: ICTROMI,},
year={2019},
pages={267-271},
publisher={SciTePress},
organization={INSTICC},
doi={10.5220/0009986302670271},
isbn={978-989-758-469-5},
}

in EndNote Style

TY - CONF

JO - Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease - Volume 1: ICTROMI,
TI - Bullous Urticaria Pigmentosa in an Infant: A Rare Form of Bullous Disorder
SN - 978-989-758-469-5
AU - Anissa L.
AU - Mahri S.
AU - Astriningrum R.
AU - Agustin T.
AU - Rihatmadja R.
AU - Rahmayunita G.
PY - 2019
SP - 267
EP - 271
DO - 10.5220/0009986302670271