medigraphic.com
ISSN 1405-1699 (Print)
Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

2021, Number 2

<< Back

Alerg Asma Inmunol Pediatr 2021; 30 (2)

Stevens Johnson/toxic epidermal necrolysis overlap syndrome associated with hypogammaglobulinemia in the neonatal period: case report

Ramírez-Vásquez DG, Julián-García O, Ramírez-Cruz L, Martínez-Canseco R

Language: Spanish
References: 11
Page: 58-61
PDF size: 232.18 Kb.


ABSTRACT

The overlap of Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is an acute, rare drug reaction associated to a mortality rate of up to 29%. According to the widely accepted classification, the SJS/TEN overlap is considered an extensive manifestation of SJS, affecting between 10-30% of the body surface, presenting in the form of blisters and erosions caused mainly by drugs and infections. Case report: The case of a 1-month-old male patient is presented; at 12 days of age, he started showing generalized dermatosis with mucosal involvement characterized by blisters and blood crusts with a histopathological report of compatible superficial perivascular lymphocyte-pauciinflammatory vacuolar interface dermatitis along toxic epidermal necrolysis. Treatment using immunoglobulin was established at 2 g/kg/do, however, he later died of septic shock secondary to Candida albicans. Conclusion: SJS/TEN overlap is considered extremely rare in the neonatal period, it has few reported cases, so it should be considered within the differential diagnosis of bullous syndromes and therefore a skin biopsy shoud be performed in a timely manner.


REFERENCES

  1. Tristani-Firouzi P, Petersen MJ, Saffle JR, Morris SE, Zone JJ. Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children. J Am Acad Dermatol. 2002; 47 (4): 548-552.

  2. Frey N, Jossi J, Bodmer M, Bircher A, Jick SS, Meier CR et al. The epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the UK. J Invest Dermatol. 2017; 137 (6): 1240-1247.

  3. McPherson T, Exton LS, Biswas S, Creamer D, Dziewulski P, Newell L et al. British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018. Br J Dermatol. 2019; 181 (1): 37-54.

  4. Lee HY, Chung WH. Toxic epidermal necrolysis: the year in review. Curr Opin Allergy Clin Immunol. 2013; 13 (4): 330-336.

  5. Demoly P, Adkinson NF, Brockow K, Castells M, Chiriac AM, Greenberger PA et al. International Consensus on drug allergy. Allergy. 2014; 69 (4): 420-437.

  6. Corrick F, Anand G. Question 2: Would systemic steroids be useful in the management of Stevens-Johnson syndrome? Arch Dis Child. 2013; 98 (10): 828-830.

  7. Lohmeier K, Megahed M, Schulte KW, Stannigel H, Mayatepek E, Schroten H. Toxic epidermal necrolysis in a premature infant of 27 weeks' gestational age. Br J Dermatol. 2005; 152 (1): 150-151.

  8. de Groot R, Oranje AP, Vuzevski VD, Mettau JW. Toxic epidermal necrolysis probably due to Klebsiella pneumoniae sepsis. Dermatologica. 1984; 169 (2): 88-90.

  9. Scully MC, Frieden IJ. Toxic epidermal necrolysis in early infancy. J Am Acad Dermatol. 1992; 27 (2 Pt 2): 340-344.

  10. Islam S, Singer M, Kulhanjian JA. Toxic epidermal necrolysis in a neonate receiving fluconazole. J Perinatol. 2014; 34 (10): 792-794.

  11. Anzilotti C, Swan DJ, Boisson B, Deobagkar-Lele M, Oliveira C, Chabosseau P et al. An essential role for the Zn2+ transporter ZIP7 in B cell development. Nat Immunol. 2019; 20 (3): 350-361.




2020     |     www.medigraphic.com