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Alergia, Asma e Inmunología Pediátricas

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2021, Number 2

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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
Full text How to cite this article 10.35366/101644

DOI

DOI: 10.35366/101644
URL: https://dx.doi.org/10.35366/101644

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


Key words:

Toxic epidermal necrolysis, Stevens Johnson syndrome, pharmacoderma, bullous syndromes, immunodeficiency.

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

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Alerg Asma Inmunol Pediatr. 2021;30