Diagnosis: DRESS syndrome (drug reaction with eosinophilia and systemic symptoms)
A 34-year-old male presented with facial edema, fever, and lymphadenopathy after starting lamotrigine for seizure management. Initial examination revealed significant systemic involvement, prompting further evaluation for a severe drug reaction. This case highlights the importance of recognizing systemic symptoms and eosinophilia in drug-induced hypersensitivity reactions.
A 34-year-old male with a history of epilepsy presented to the emergency department with facial edema, high-grade fever, and generalized lymphadenopathy. Symptoms began approximately three weeks after initiating lamotrigine therapy. On examination, he exhibited significant facial swelling, conjunctival injection, and cervical lymphadenopathy.Facial edema: Marked swelling, particularly around the eyes.Fever: Sustained temperature of 39.5°C (103.1°F).Lymphadenopathy: Enlarged cervical and axillary lymph nodes.Rash: Erythematous macules and papules on the trunk and extremities.Eosinophilia: Laboratory results revealed a peripheral eosinophil count of 1500 cells/µL.
The patient experienced a gradual onset of symptoms three weeks after starting lamotrigine, which was initiated for seizure control. He had no prior history of drug allergies and no significant family history of dermatologic conditions. He reported occasional use of over-the-counter analgesics but had not taken any other new medications recently. The patient denied any recent infections or travel history.Onset: Symptoms began three weeks post initiation of lamotrigine.Triggers: Recent medication change with lamotrigine.Past medical history: Epilepsy, well-controlled on current regimen.Family history: No known drug allergies or autoimmune diseases.Social history: Non-smoker, no alcohol use, works as a software engineer.
Acute / First-Line ManagementImmediate discontinuation of the offending agent (lamotrigine).Initiation of systemic corticosteroids: Prednisone 1 mg/kg/day for 1-2 weeks, with gradual taper based on clinical response.Supportive care, including hydration and antipyretics for fever.Workup and Diagnostic ConfirmationComplete blood count (CBC) to assess eosinophilia and leukocyte count.Liver function tests (LFTs) to evaluate for hepatic involvement.Renal function tests to assess kidney involvement.Skin biopsy may be considered if the diagnosis remains uncertain.Long-Term ManagementMonitor for recurrence of symptoms after corticosteroid taper.Consider skin and systemic evaluations for any long-term sequelae.Educate the patient regarding avoidance of known allergens and the importance of informing healthcare providers of this severe reaction in the future.
Stevens-Johnson Syndrome (SJS): Characterized by mucosal involvement and widespread skin sloughing; usually occurs within 1-3 weeks of drug exposure.Acute Generalized Exanthematous Pustulosis (AGEP): Presents with pustular eruption and fever, typically within days of exposure to certain medications.Fixed Drug Eruption (FDE): Localized lesions that recur at the same site upon re-exposure to the drug; often not associated with systemic symptoms.Drug-Induced Hypersensitivity Syndrome (DIHS): Similar to DRESS but often has a shorter onset time and distinct clinical features such as fever and lymphadenopathy.Viral exanthems: Common infections (e.g., EBV, CMV) can mimic drug reactions with systemic symptoms like fever and lymphadenopathy.Systemic Lupus Erythematosus (SLE): Consider in patients with systemic symptoms and eosinophilia, especially if there's a history of photosensitivity or arthralgias.Vasculitis: May present with systemic symptoms and eosinophilia; consider if there are additional systemic features.Dermatitis herpetiformis: Typically associated with celiac disease; presents with pruritic papules and vesicles.
High-Yield PearlsRecognition: Early identification of severe cutaneous adverse drug reactions is crucial for patient management and can prevent life-threatening complications.Eosinophilia: Presence of eosinophilia in conjunction with systemic symptoms should raise suspicion for DRESS syndrome.Drug history: A detailed medication history is essential to identify potential triggers, particularly in patients starting new therapies.Corticosteroids: Systemic corticosteroids are the mainstay of treatment for DRESS syndrome, with a typical starting dose of prednisone at 1 mg/kg/day.Monitoring: Close monitoring during corticosteroid tapering is necessary to detect any recurrence of symptoms.Understanding the nuances of drug reactions can significantly impact patient outcomes and management strategies.
Tags: DRESS, drug reaction