Drug rash with eosinophilia and systemic symptoms syndrome

Background

Known as DRESS syndrome
A severe adverse drug reaction
Usually begins within 8 weeks of starting a new drug
8-10% mortality
Previously known as Dilantin Hypersensitivity Syndrome or Anti-convulsant hypersensitivity syndrome
However, many other medications, particularly antibiotics
A virus-drug interaction with HHV-6, HHV-7, EBV, and CMV may be a form of pathogenesis as well^[1]

DRESS induced by chloral hydrate: (A) Generalized edematous features & systemic morbilliform rash (B) Sternostomy wounds (C) hepatosplenomegaly.

Allopurinol causing DRESS: Rash spreading symmetrically to the lower extremities (non-blanching).

DRESS due to anti-TB medication.

Fever and rash (varying; may resemble SJS) are typically first signs
- Usually urticarial, maculopapular eruption
- In some cases vesicles, bullae, pustules, purpura, target lesions, facial edema, cheilitis, erythroderma
+/- lymphadenopathy
Systemic involvement may manifest as hepatitis, pneumonitis, myocarditis, pericarditis, nephritis, colitis
- Atypical lymphocytes, thrombocytopenia, neutropenia, anemia
- Leukocytosis with eosinophilia in 50-90%, and mononucleosis in 40%^[3]^[4]

Charateristic	DRESS	SJS/TEN	AGEP	Erythroderma
Image
Onset of eruption	2-6 weeks	1-3 weeks	48 hours	1-3 weeks
Duration of eruption (weeks)	Several	1-3	<1	Several
Fever	+++	+++	+++	+++
Mucocutaneous features	Facial edema, morbilliform eruption, pustules, exfoliative dermattiis, tense bullae, possible target lesions	Bullae, atypical target lesions, mucocutaneous erosions	Facial edema, pustules, tense bullae, possible target lesions, possibl emucosal involvement	Erythematous plaques and edema affecting >90% of total skin surface with or without diffuse exfoliation
Lymph node enlargement	+++	-	+	+
Neutrophils	Elevated	Decreased	Very elevated	Elevated
Eosinophils	Very elevated	No change	Elevated	Elevated
Atypical lymphocytes	+	-	-	+
Hepatitis	+++	++	++	-
Other organ involvement	Interstitial nephritis, pneumonitis, myocarditis, and thydoiditis	Tubular nephritis and tracheobronical necrosis	Possible	Possible
Histological pattern of skin	Perivascular lymphocytcic infiltrate	Epidermal necrosis	Subcorneal pustules	Nonspecific, unless reflecting Sezary syndrome or other lymphoma
Lymph node histology	Lymphoid hyperplasia	-	-	No, unless reflecting Sezary syndrome or other malignancy
Mortality (%)	10	5-35	5	5-15

Discontinue suspected drug
Supportive care: antipyretic, anti-pruritic
Fluid management as in burn
Systemic steroids and/or cyclosporin/immunosuppressants in severe cases (controversial)
- Hepatitis, pneumonitis, extensive exfoliative dermatitis
- Other severe systemic manifestations
Family counseling as possible genetic component

↑ Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. Sonal Choudhary, Michael McLeod, Daniele Torchia, Paolo Romanelli. J Clin Aesthet Dermatol. 2013 Jun; 6(6): 31–37.
↑ Herman AO. Antipsychotic Linked to Potentially Fatal Skin Reaction. Physician's First Watch. Dec 12, 2014. http://www.jwatch.org/fw109630/2014/12/12/antipsychotic-linked-potentially-fatal-skin-reaction?query=pfw&jwd=000013530619&jspc=.
↑ Callot V, Roujeau JC, Bagot M, et al. Drug-induced pseudolymphoma and hypersensitivity syndrome. Two different clinical entities. Arch Dermatol. 1996;132:1315–1321.
↑ Peyriere H, Dereure O, Breton H, et al. Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist? Br J Dermatol. 2006;155:422–428.

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