MKSAP Quiz: generalized rash, facial edema, fever, and severe fatigue
MKSAP Quiz: generalized rash, facial edema, fever, and severe fatigue
A 51-year-old woman is evaluated for generalized rash, facial edema, fever, and severe fatigue that have developed over the past week. She was recently diagnosed with rheumatoid arthritis. She currently takes prednisone, hydroxychloroquine, and sulfasalazine. She has no previously known allergies.
On physical examination, temperature is 39.1 °C (102.3 °F), blood pressure is 110/78 mm Hg, pulse rate is 108/min, and respiration rate is 25/min. The face is edematous. Skin examination reveals a generalized morbilliform eruption. The skin is not painful, and no blisters are present. There is no ocular or mucosal involvement. Lymphadenopathy is noted in the cervical and axillary regions. Laboratory studies show a serum alanine aminotransferase level of 330 U/L and a serum aspartate aminotransferase level of 355 U/L. Results of a complete blood count are normal except for 16% eosinophils.
Which of the following is the most likely diagnosis?
A. Drug reaction with eosinophilia and systemic symptoms (DRESS)
B. Erythema multiforme
C. Stevens-Johnson syndrome
D. Toxic epidermal necrolysis
MKSAP Answer and Critique
The correct answer is A) Drug reaction with eosinophilia and systemic symptoms (DRESS). This item is available to MKSAP 15 subscribers as item 4 in the Dermatology module.
This patient has a drug reaction characterized by a generalized papular eruption, eosinophilia, and systemic symptoms and is consistent with drug reaction with eosinophilia and systemic symptoms (DRESS). With cessation of the causative drug, most likely sulfasalazine, the skin reaction rapidly subsides along with lymphadenopathy, fever, elevated aminotransferase levels, and eosinophilia. Patients with DRESS may develop severe hepatitis, and fulminant hepatic necrosis may occur if the condition is unrecognized.
Erythema multiforme (EM) is an acute, often recurrent mucocutaneous eruption that usually follows an acute infection, most frequently recurrent herpes simplex virus infection, but it may also be drug related or idiopathic. Lesions range in size from several millimeters to several centimeters and consist of erythematous plaques with concentric rings of color. Patients may have low-grade fever during an EM outbreak. However, this patient's skin lesions are not consistent with EM, and EM does not cause lymphadenopathy, aminotransferase elevations, or eosinophilia.
The reactions that are generally classified within the spectrum of severe cutaneous adverse reactions include acute generalized exanthematous pustulosis, Stevens-Johnson syndrome/toxic epidermal necrolysis, DRESS, and vasculitis. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are characterized by fever, skin pain, and mucocutaneous lesions resulting in epidermal death and sloughing. The clinical difference between SJS and TEN is the severity and percentage of body surface involved. SJS involves less than 10% of body surface area, SJS/TEN overlap involves 10% to 30%, and TEN involves greater than 30%. This patient's skin lesions are not compatible with SJS or TEN, and these conditions cannot explain the patient's other systemic findings.
Key Point
- Drug reaction with eosinophilia and systemic symptoms (DRESS) is a serious cutaneous adverse reaction characterized by a generalized papular eruption, facial edema, fever, arthralgia, and generalized lymphadenopathy and is commonly associated with elevated aminotransferase levels, eosinophilia, and lymphocytosis.