Diffuse Cutaneous Mastocytosis and Vaccination Reactions in a Filipino Child: A Case Report

Abstract

Introduction: Diffuse cutaneous mastocytosis (DCM) is a rare and severe form of cutaneous mastocytosis which may present in the neonatal period; thus early recognition is essential. Symptoms of mastocytosis are exacerbated by mast cell degranulating agents more commonly from heat, friction, local trauma, drugs and food. This is a case of DCM presenting with bullous eruptions after immunization.

Case Report: An 11-month-old boy presented with generalized erythematous to hyperpigmented macules and patches initially at birth, with progression to bullous eruptions immediately after immunization without any systemic symptoms. Biopsy revealed superficial and deep mixed cell infiltrates consisting of lymphocytes, histiocytes and numerous mast cells. Giemsa stain highlighted the metachromatic mast cell granules. Serum tryptase was elevated by 13 times (130 ug/L). The patient was prescribed oral antihistamines and topical steroids that offered good response. Avoidance of all potential triggers was instructed.

Discussion: The extensive cutaneous involvement in DCM (generalized erythema, diffuse papules that develop pachyderma, darker skin, peau d’orange) are due to the diffuse infiltration of the dermis with mast cells, accompanied with an elevated serum tryptase.

Unique to this local case are exacerbations triggered by vaccination. There is literature to support evidence of vaccination reactions to pentavalent vaccine in children with DCM though the pathway associated with mast cell degranulation after immunization has not yet been specified.

It is advised that patients with DCM follow scheduled immunization guidelines with precautionary measures.

Key Words: Diffuse cutaneous mastocytosis, mastocytosis, immunizations, vaccines

  1. Petersone G, Zigure S, Zavorins A. Diffuse cutaneous mastocytosis. Ann Clin Case Rep [Internet]. 2018;3:1511. Available from: https://www.anncaserep.com/full-text/accr-v3-id1511.php. 
  2. Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood [Internet]. 2017;129(11):1420–7. Available from: http://dx.doi.org/10.1182/blood-2016-09-731893 
  3. Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, 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 [Internet]. 2016;137(1):35–45. Available from: http://dx.doi.org/10.1016/j.jaci.2015.08.034 
  4. Patrizi A, Virdi A, Neri I. Cutaneous mastocytosis exacerbated by pinworms in a young boy: Brief report. Pediatr Dermatol [Internet]. 2012;29(2):229–30. Available from: http://dx.doi.org/10.1111/j.1525-1470.2011.01530.x 
  5. Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin North Am [Internet]. 2014;34(2):283–95. Available from: http://dx.doi.org/10.1016/j.iac.2014.01.003 
  6. Ke BN, Abalos-Babaran S, Barit J-VJG, Gervacio MKR, Ramirez-Quizon MN. Bullous mastocytosis in a Filipino infant: a case study. Acta Med Philipp [Internet]. 2021;55(5). Available from: http://dx.doi.org/10.47895/amp.v55i5.2326 
  7. Hosking A-M, Makdisi J, Ortenzio F, de Feraudy S, Smith J, Linden K. Diffuse cutaneous mastocytosis: Case report and literature review. Pediatr Dermatol [Internet]. 2018;35(6):e348–52. Available from: http://dx.doi.org/10.1111/pde.13651 
  8. Bankova LG, Walter JE, Iyengar SR, Lorenzo ME, Hornick JL, Castells MC. Generalized bullous eruption after routine vaccination in a child with diffuse cutaneous mastocytosis. J Allergy Clin Immunol Pract [Internet]. 2013;1(1):94–6. Available from: http://dx.doi.org/10.1016/j.jaip.2012.08.008 
  9. Theoharides TC, Valent P, Akin C. Mast cells, mastocytosis, and related disorders. N Engl J Med [Internet]. 2015;373(2):163–72. Available from: http://dx.doi.org/10.1056/NEJMra1409760 
  10. Sarcina D, Giovannini M, Oranges T, Barni S, Pedaci FA, Liccioli G, et al. Case report and review of the literature: Bullous skin eruption after the booster-dose of influenza vaccine in a pediatric patient with polymorphic maculopapular cutaneous mastocytosis. Front Immunol [Internet]. 2021;12:688364. Available from: http://dx.doi.org/10.3389/fimmu.2021.688364 
  11. Johansen ML, Lawley LP. Assessing vaccination reactions in pediatric patients with maculopapular cutaneous mastocytosis. Pediatr Dermatol [Internet]. 2021;38(2):502–3. Available from: http://dx.doi.org/10.1111/pde.14492 

Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License, which permits use, share — copy and redistribute the material in any medium or format, adapt — remix, transform, and build upon the material, as long as you give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use. You may not use the material for commercial purposes. If you remix, transform, or build upon the material, you must distribute your contributions under the same license as the original. You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit https://creativecommons.org/licenses/by-nc-sa/4.0/.