DRESS syndrome and severe cutaneous reactions

DRESS syndrome and severe cutaneous reactions

DRESS syndrome and severe cutaneous reactions

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SCAR Spectrum - Defining The Danger

  • Stevens-Johnson Syndrome (SJS): Widespread blisters and mucosal involvement with skin detachment of <10% Body Surface Area (BSA).
  • SJS/TEN Overlap: A transitional form with 10-30% BSA detachment.
  • Toxic Epidermal Necrolysis (TEN): The most severe form, with >30% BSA detachment and positive Nikolsky's sign.
  • DRESS Syndrome: Characterized by a long latency period (2-8 weeks), fever, extensive rash, eosinophilia, and systemic organ failure (especially liver, kidneys).

SJS vs. TEN: Epidermal Detachment Comparison

⭐ TEN carries a significantly higher mortality rate (>30%) compared to SJS (~5-10%), primarily due to sepsis from the loss of the skin barrier.

Causative Drugs - The Usual Suspects

  • Anticonvulsants (Aromatic):
    • Carbamazepine, Lamotrigine, Phenytoin, Phenobarbital
  • Allopurinol:
    • High risk, especially with renal impairment or diuretic use.
  • Sulfonamides:
    • TMP-SMX, Sulfasalazine
  • Antibiotics:
    • Minocycline, Vancomycin
  • Antiretrovirals:
    • Abacavir (test for HLA-B*57:01), Nevirapine

High-Yield: Allopurinol-induced SCARs show a strong association with the HLA-B*58:01 allele, particularly in Han Chinese, Thai, and Korean populations. Consider genetic screening in these groups before initiating therapy.

Path & Presentation - Code Red Reaction

  • Pathogenesis: A severe, delayed Type IVb hypersensitivity reaction. Involves drug-activated T-cells releasing cytokines (e.g., IL-5), leading to eosinophil recruitment. Frequently associated with reactivation of latent viruses like HHV-6 and EBV.

  • Clinical Triad:

    • Latency: Long delay of 2-8 weeks between drug initiation and symptom onset.
    • Rash: Diffuse, morbilliform (measles-like) eruption that can become confluent, vesicular, or exfoliative. Prominent facial edema is highly characteristic.
    • Systemic Involvement: High fever, widespread lymphadenopathy, and multi-organ inflammation.
  • Lab Findings:

    • Eosinophilia (>1500/μL) and atypical lymphocytes are hallmark findings.
    • Elevated liver enzymes indicate hepatitis, the most common visceral complication.

⭐ Myocarditis is a less common but highly lethal complication of DRESS syndrome, mandating a high index of suspicion in patients with chest pain or ECG changes.

Diagnosis & Action - Damage Control Protocol

  • Initial Steps:

    • High index of suspicion is crucial.
    • IMMEDIATELY withdraw the offending drug.
    • Supportive care: fluids, electrolytes, nutrition.
  • Confirmation:

    • Labs: CBC (↑ eosinophils, atypical lymphocytes), ↑ LFTs, ↑ creatinine.
    • Skin biopsy (punch): Confirms diagnosis (perivascular lymphocytic infiltrate, eosinophils).
  • Treatment Algorithm:

Exam Favorite: DRESS syndrome is strongly associated with the reactivation of Human Herpesvirus 6 (HHV-6), which can contribute to the systemic inflammation and organ damage.

  • DRESS syndrome is a drug reaction with eosinophilia and systemic symptoms, presenting with rash, fever, and internal organ (liver, kidney) damage after a long latency (2-8 weeks).
  • Key triggers include allopurinol, anticonvulsants (phenytoin, carbamazepine), and sulfonamides.
  • SJS/TEN are severe mucocutaneous reactions with widespread blistering, epidermal necrosis, and mucosal involvement.
  • Distinguished by BSA detachment: SJS <10%, SJS-TEN overlap 10-30%, and TEN >30%.
  • Management for all hinges on immediate withdrawal of the offending drug; this is the most critical step.
  • Specific HLA alleles (e.g., HLA-B*1502 with carbamazepine) are major risk factors.
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Practice Questions: DRESS syndrome and severe cutaneous reactions

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A 10-year-old boy presents with a painful rash for 1 day. He says that the reddish, purple rash started on his forearm but has now spread to his abdomen. He says there is a burning pain in the area where the rash is located. He also says he has had a stuffy nose for several days. Past medical history is significant for asthma and epilepsy, medically managed. Current medications are a daily chewable multivitamin, albuterol, budesonide, and lamotrigine. On physical examination, there is a red-purple maculopapular rash present on upper extremities and torso. There are some blisters present over the rash, as shown in the image, which is also present in the oral mucosa. Which of the following is the most likely cause of this patient’s symptoms?

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Flashcards: DRESS syndrome and severe cutaneous reactions

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Sulfa drugs are associated with the rare cutaneous skin complication _____

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Sulfa drugs are associated with the rare cutaneous skin complication _____

Steven-Johnson syndrome

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