Maculopapular cutaneous Mastocytosis

Authors

  • Crisbety Soares Pinho Department of Pediatrics, Centro Hospitalar e Universitário de Coimbra
  • Inês Barreto Department of Pediatrics, Centro Hospitalar e Universitário de Coimbra
  • Sandra Rebimbas Department of Pediatrics, Centro Hospitalar do Baixo Vouga https://orcid.org/0000-0002-8286-4026
  • Sónia Coelho Department of Dermatology, Centro Hospitalar Tondela Viseu https://orcid.org/0000-0001-5241-7017

DOI:

https://doi.org/10.25753/BirthGrowthMJ.v31.i1.23848

Keywords:

Darier, mastocitose, Pediatrics

Abstract

Mastocytosis is a rare disorder caused by proliferation and accumulation of mast cells in various tissues, with characteristic symptoms associated with the release of their mediators. Its cutaneous form, usually benign, is the most frequent among children.

The authors report the case of a two-month-old male infant who presented to the Emergency Department with small, tan-to-brown macules and papules distributed mainly on the trunk, which progressively became nodular and bullous, with one month of evolution. Darier´s sign was positive.

The absence of systemic signs and analytic (including serum tryptase level) and echographic changes was consistent with the diagnosis of maculopapular cutaneous mastocytosis, or urticaria pigmentosa, subsequently confirmed by skin biopsy.

Treatment with anti-histaminic therapy and topical immunosuppressant and avoidance of triggering factors led to a positive outcome.

Downloads

Download data is not yet available.

Author Biography

Crisbety Soares Pinho, Department of Pediatrics, Centro Hospitalar e Universitário de Coimbra

Interna de Pediatria do Hospital Pediátrico de Coimbra

References

Castells M, Metcalfe D, Escribano L. Guidelines for the Diagnosis and Treatment of Cutaneous Mastocytosis in Children. Am J Clin Dermatol 2011; 12(4): 259–70.

Tamay Z, Özçeker D. Current approach to cutaneous mastocytosis in childhood. Turk Pediatri Ars 2016; 51(3): 123–7.

Pardanani A. CME Information: Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management. Am J Hematol 2016; 91(11): 1146–59.

Méni C, Bruneau J, Georgin-Lavialle S, Le Saché de Peufeilhoux L, Damaj G, Hadj-Rabia S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 2015; 172(3): 642–51.

Hartmann K, Escribano L, Grattan C, Brockow K, Carter M. 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.

Klaiber N, Kumar S, Irani A-M. Mastocytosis in Children. Curr Allergy Asthma Rep 2017; 17(80).

Tarjuelo DV, Majlis PZ, Ortiz LB, Hermosa JMH. Mastocitosis cutánea. Acta Pediatr Esp 2007; 65(6): 282–5.

Valent P, Aki C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood 2017; 129(11): 1420–7.

Matito A, Azaña JM, Torrelo A, Alvarez-Twose I. Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors. Immunol Allergy Clin North Am 2018; 38(3): 351–63.

Tharp MD. Mastocytosis. In: Kang S, Amagai M, Bruckner A L, Enk A H, Margolis D J, McMichael A J, Orringer J S, editors. Fitzpatrick´s Dermatology in General Medicine. Ninth edition. McGraw-Hill Education; 2019. p. 710-22.

Alvarez-Twose I, Vañó-Galván S, Sánchez-Muñoz L, Morgado JM, Matito A, Torrelo A, et al. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012; 67(6): 813–21.

Carter MC, Clayton ST, Komarow HD, Brittain EH, Scott LM, Cantave D, et al. Assessment of clinical findings, tryptase levels, and bone marrow histopathology in the management of pediatric mastocytosis. J Allergy Clin Immunol 2015; 136(6): 1673–9.

Alemán GM, El Habr C, Norris DI, Dámaso TM, Borrego L, Sangueza OP. Cutaneous Mastocytosis With Atypical Mast Cells in a 7-Year-Old Girl. Am J Dermatopathol 2017; 39(4): 310–2.

Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, et al. Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria. Eur J Clin Invest 2007; 37(6): 435–53.

Mashiah J, Harel A, Bodemer C, Hadj-Rabia S, Goldberg I, Sprecher E, et al. Topical pimecrolimus for paediatric cutaneous mastocytosis. Clin Exp Dermatol 2017; 43(5): 559–65.

Vaz AS, Rodrigues J, Moura L, Mascarenhas R. Early diagnosis of cutaneous mastocytosis in an infant: the importance of a clinical sign. BMJ Case Rep 2019; 12(1).

Downloads

Published

2022-04-04

How to Cite

1.
Pinho CS, Barreto I, Rebimbas S, Coelho S. Maculopapular cutaneous Mastocytosis. REVNEC [Internet]. 2022Apr.4 [cited 2022May26];31(1):68-72. Available from: https://revistas.rcaap.pt/nascercrescer/article/view/23848

Issue

Section

Case Reports