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RESIDENT REPORT
Sonya Dasharathy, PGY-2, UCLA Internal Medicine
HPI
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55 yo M with h/o ESRD s/p DDRT in 2005 with
rejection in 6/2015 on HD MWF via L permacath,
CAD s/p 3v CABG in 10/2015, HTN, pAF on AC,
gout
Recently admitted to CCU 10/23-11/12 for chest
pain and found to have 3v CAD. Was taken to OR
for CABG. Also found to have severe PAD and
treated for infection with ceftriaxone->Keflex
Discharged on 11/12 with f/u with vascular for
PAD.
HPI
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Developed malaise, rash that started on trunk and
spread to upper extremities and then lower
extremities over last three days; not palpable and
pruritic
Also fevers, chils, malaise, fatigue, pain in RLE with
erythema decreased ambulation
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PMH
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Aellrgies: NKDA
Social Hx
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ESRD s/p DDRT in 2005
CAD s/p 3v CABG in
10/2015
HTN
pAF
Gout
single lives with parents,
brother, and nephew – works
as warehouse receiver, 1 can
beer/week, 0.5packs/day x
31 years – quit in 8/2015
Family Hx: non-contributory
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Medications
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Allopurinol
Amiodarone
Aspirin
Atorvastatin
Calcitriol
Colchicine
Gabapentin
Metoprolol
Omeprazole
Warfarin
Sevelamer
s/p ceftriazone/keflex 3
weeks ago
Discussion
Rash Nomenclature
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Macule: Circumscribed area of change in normal skin color, with no
skin elevation or depression; may be any size
Papule: Solid, raised lesion up to 0.5 cm in greatest diameter
Plaque: Elevation of skin occupying a relatively large area in
relation to height; often formed by confluence of papules
Vesicle: Circumscribed, elevated, fluid-containing lesion less than 0.5
cm in greatest diameter; may be intraepidermal or subepidermal in
origin
Bulla: Same as vesicle, except lesion is more than 0.5 cm in greatest
diameter
Pustule: Circumscribed elevation of skin containing purulent fluid of
variable character (i.e., fluid may be white, yellow, greenish or
hemorrhagic)
Maculopapular
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Viruses
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lyme disease, RMSF,
ehrlichiosis
Autoimmune
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scarlet fever, toxic shock,
secondary syphilis
Tick Borne
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measles, rubella, roseola,
HIV, erythema infectiosum
Kawasaki, RA, SLE
Drug reaction
Localize
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Bacteria
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Erythema infectiosum,
erythema migrans, cellulitis,
scabies, insect bites
Diffuse
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Measles, rubella, roseola,
scarlet fever, toxic shock,
kawasaki
Vesicular/bullose/pustular
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Virus:
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 HSV,
 HSV,
zoster, coxackie,
impetigo
VZV, coxackieviruses
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Bacteria:
 staph/impetigo
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Other:
 TEN/SJS
Localized
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Diffuse
 SJS,
TEN, staph
scalded skin syndrome,
varicella
Back to the case…
Physical Exam
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VS: 38.5, 112, 19, 110/62, 98% on RA
HEENT: Normocephalic, atraumatic, EOMI, normal conjunctivae, no scleral icterus,
clear oropharynx, poor dentition; mild bilateral facial swelling
CV: grade II/VI holosystolic murmur
Chest: L permacath site c/d/i, nontender; sternotomy scar visible
Pulm: Normal respiratory effort, CTA bilaterally, no wheezes, rales, or rhonchi
Abd: Normoactive bowel sounds, soft, nontender, nondistended.
Ext: Dry gangrene of R great toe with no warmth. Erythema of remaining R toes.
BL DP and TP pulses non-palpable but Dopplerable. Some overlying
erythema/warmth
LN: diffuse lymphadenopathy
Skin: morbilliform rash over trunk, back, and upper/lower extremities with
desquamation
Neuro: Alert and oriented, moves all extremities spontaneously except for
diminished strength in R toes
Basic Labs
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CBC: 6.68/8.5/206
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60.9% neutrophils, 19% eosinophils
BMP: 126/4.9/98/26/17/5.3
LFTS: AST 68, ALT 91, ALP 118, TB 0.4, TP 6.1, Alb 3.6
ESR 56, CRP 20.5
Lactate 8 (nml), CK 70
Coags WNL except for INR 2-3
Urinalysis 3+ blood, neg nitrite/LE, RBC > 1k, WBC 1k,
marked urine eos
Micro
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RPR negative, hIV negative
CMV HHV6, parvo, BK virus, EBV neg
Cultures drawn; blood and urine negative
eventually
DIFFERENTIAL DIAGNOSIS
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SJS/TEN
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Hypereosinophilic syndromes
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NAACP:
 Neoplasms
 Addison’s disease
 Allergic diseases
 Collagen vascular disease
 Parasitic diseases
PLUS….
 Eosinophilic GI diseases
Mild eosinophilia = more likely asthma or allergic rhinitis; very severe
eosinophilia (≥20,000) more likely myeloproliferative neoplasm
Rest of the case…
DRUG REACTION WITH EOSINOPHILIA
AND SYSTEMIC SYSTEMS (DRESS)
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Rare, potentially life-threatening, drug-induced
hypersensitivity reaction
Includes skin eruption, hematologic abnormalities
(eosinophilia, atypical lymphocytosis),
lymphadenopathy, and internal organ involvement
(liver, kidney, lung)
Long latency (2-8 weeks) between drug exposure
and disease onset
Prolonged course with frequent relapses despite the
discontinuation of the culprit drug
EPIDEMIOLOGY
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Incidence is unknown
Frequency varies depending upon the type of drug
and immune status of the patient
ETIOLOGY AND RISK FACTORS
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Drug causality is determined as "highly probable" in
approximately 80%
In10-20% of cases fulfilling the diagnostic criteria for DRESS,
a relationship with a drug cannot be established
Antiepileptic
agents(eg, carbamazepine, lamotrigine, phenytoin, phenobarbi
tal) and allopurinol are the most frequently reported causes
Sulfonamides (particularly sulfasalazine), dapsone, minocycline,
and vancomycin
Several cases of DRESS have been attributed to raltegravir
and vemurafenib
Pharmacogenetic studies have found an association between HLA
haplotypes and susceptibility to DRESS
PATHOGENESIS
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Drug-specific immune response and herpesvirus
reactivation are key factors
Type IV hypersensitivity reaction
Th2 immune reaction: cells secrete cytokines, such as
IL-5 which leads to B cell production of IgE,
macrophage deactivation, eosinophilic inflammation
Expansion of regulatory T cells may contribute to
herpesvirus reactivation
CLINICAL PRESENTATION
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Systemic symptoms: fever, malaise, diffuse
lymphadenopathy
Symmetric facial edema with erythema
Organ involvement: liver, kidney, lung
Laboratory abnormalities:
Leukocytosis with eosinophil counts > 700
 Atypical lymphocytosis
 Abnormal LFTs; especially elevated ALT
 Elevated Cr, low grade proteinuria, and abnormal urinary
sediment with occasional eosinophils
 HHV-6 infection
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CLINICAL PRESENTATION
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Starts as a morbilliform eruption that progresses to a diffuse,
confluent, and infiltrated erythema with follicular accentuation
RegiSCAR SCORE CALCULATOR
Kardaun et al, Br J Derm 2007 and Cacoub et al, Am J Med 2011.
MANAGEMENT
Withdrawal of offending drug! (plus
avoidance of new medications)
 IVF, electrolyte/nutritional support if needed
 Allergy/Immunology and Dermatology consult
 WITHOUT severe organ involvement:
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 Symptomatic
relief: high potency topical
corticosteroids 2-3x/day x 1 week
MANAGEMENT
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WITH severe organ involvement:
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Liver:
 Systemic corticosteroids are of unproven benefit
 May require liver transplantation
Lung or kidney:
 Systemic corticosteroids (minimum of 0.1mg/kg prednisone) until
clinical improvement and normalization of labs; tapered over 8-12
weeks
 Has not been evaluated in RCTs
No studies evaluating the treatment of DRESS with antiviral agents
active against HHV6 or CMV
IVIG has been effective in some cases that do not respond to systemic
steroids
MANAGEMENT
Husain X et al, . J Am Acad Dermatol. 2013
PROGNOSIS
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Most patients recover completely in weeks to months
after drug withdrawal but can relapse with too
rapid steroid tapering
Autoimmune diseases have been reported
months/years after resolution
Increased risk of reaction to structurally unrelated
drugs
Retrospective studies reported mortality rate of 510%
REFERENCES
1.
2.
3.
4.
5.
Husain Z, Reddy BY, Schwartz RA. DRESS syndrome: Part I. Clinical
perspectives. J Am Acad Dermatol. 2013; 68:693.e1.
Cacoub P, Musette P, Descamps V, et al. The DRESS syndrome: a
literature review. Am J Med. 2011 Jul;124(7):588-97
Kardaun SH, Sidoroff A, Valeyrie-Allanore L, 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.
2007;156:609-611
Funck-Brentano E, Duong TA, Bouvresse S, et al. Therapeutic
management of DRESS: a retrospective study of 38 cases. J Am
Acad Dermatol. 2015;72(2):246.
Husain X, Reddy BY, Schwarz RA. DRESS syndrome: Part II.
Management and therapeutics. J Am Acad Dermatol. 2013
May;68(5):709.