DRESS TO TEN: IS THIS A CONTINUUM?

Publication date: November 2018

Source: Annals of Allergy, Asthma & Immunology, Volume 121, Issue 5, Supplement

Author(s): F. Khan, J. Simonaire, N. Klaiber, S. Kumar

Introduction

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a potentially life-threatening severe cutaneous adverse drug reaction (SCAR) that can be triggered by medications. Toxic Epidermal Necrolysis (TEN) is also a type of SCAR. To the best of our knowledge, we present a first known clear case of DRESS which progressed to clinical TEN and was successfully treated.

Case Description

A 17-year old female was started on Lamotrigine and Escitalopram for a mood disorder. One week later, she developed tender cervical lymphadenopathy, fevers, and abdominal discomfort. About 2.5 weeks after starting these two medications, she developed a raised, pruritic, erythematous rash on her arms. Although the medication was discontinued, she failed to improve and developed facial angioedema, hepatic failure, eosinophilia and atypical lymphocytosis - all concerning signs for DRESS. Skin biopsy confirmed perivascular lymphocytic infiltrate with scattered eosinophils supporting DRESS. She was treated with intravenous immunoglobulin and eventually discharged home with a resolving rash. As Prednisone was tapered, the patient was readmitted with concerns for relapsing DRESS. She went on to develop TEN with mucosal involvement as well as greater than 30 percent skin involvement. She was successfully treated with a combination of Cyclosporine and high dose Prednisone.

Discussion

There is paucity of literature on SCAR overlap syndromes and we acknowledge the ambiguity and difficulty with this diagnosis given the overlapping presentation with SCAR syndromes. Although the mortality rate tends to be quite high for SCAR overlap syndromes, our patient was successfully treated with a combination of IVIG, steroids and Cyclosporine.

Progression towards TEN.

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