Morning Report

Ashley Ellsworth, MD PGY-2 April 1, 2011

DRESS Syndrome


 

Drug Reacton/Rash with Eosinophilia and Systemic Symptoms HSS: Hypersensitivity Syndrome DIHS: Drug-Induced Hypersensitivity Syndrome DIDMOHS: Drug-Induced Delayed Multiorgan Hypersensitivity Syndrome

Presentation


 

 

Maculopapular rash developing 2-6 weeks after starting the medication. Fever Leukocyte abnormalities: Leukocytosis, atypical lymphocytosis, eosinophilia Liver or other organ abnormalities Lymphadenopathy

Common causes


Anticonvulsants
   

Carbamazepine Phenytoin Phenobarbital Lamotrigine

     

Minocycline Allopurional Dapsone Abacavir Nevirapine Sulfonamides

Organ involvement
    

Liver 80% Kidney 40% Pulmonary 33% Cardiac/muscular 15% Pancreas 5%

Hematologic abnormalities
    

Atypical lymphocytes 63% Eosinophilia 52% Lymphocytopenia 45% Thrombocytopenia 25% Lymphocytosis 25%

Diagnosis


RegiSCAR inclusion criteria:

      

Hospitalization Reaction suspected to be drug-related Acute rash Fever>38 Lymphadenopathy in at least two sites Involvement of at least one internal organ Blood count abnormalities (lymphopenia, lymphocytosis, eosinophilia, thrombocytopenia)

Diagnosis


Japanese consensus group for DIHS

        

Maculopauplar rash developing > 3 wks after starting the suspected drug Prolonged symptoms 2 wks after stopping the drug Fever >38 Liver abnormalities or other organ involvement Leukocyte abnormalities Leukocytosis Atypical lymphocytosis Lymphadenopathy Human herpesvirus 6 reactivation

Diagnosis
   

Clinical diagnosis. Broad differential. Differing criteria for diagnosis. Biopsy can help, but is not necessary or specific.

Treatment
 

Discontinue offending drug Symptomatic therapy

 

Antihistamines Topical steroids Help with symptoms Unknown if alters long-term prognosis No randomized trials

Systemic corticosteroids
  

IVIG

Prognosis
   

Varied Complete resolution Liver failure, requiring transplantation Death



10% mortality

Pathogenesis
 

Unknown Genetic predisposition



HLA related genes Accumulation of harmful metabolites

Failure of drug detoxification



Drug-induced hypogammaglobulinemia followed by viral reactivation



Association with HHV6

Sources


 

Seth, Divya MD, Kamat, Deepak MD, and Jenny Montejo, MD. DRESS Syndrome: A Practical Approach for Primary Care Practitioners. Clinical Pediatrics. 2008; 47:947952. Tables obtained from Wikipedia: (Originals from Chen YC, Chiu HC, Chu C?Y.Drug Reaction with Eosiniphilia and Systemic Symptoms: A retrospective study of 60 cases. Arch Dermatol. 2010; 146: 1373-1379). Up-to-date. Drug allergy: Classification and clinical features. Walsh, SA and D Creamer. Drug reaction with eosinophilia and systemic symtpoms: a clinical update and review of current thinking. Clinical Dermatology. 2010; 36:6-11.