WHEN AGEP MET DRESS: A UNIQUE OVERLAP OF SEVERE CUTANEOUS ADVERSE REACTIONS

Document Type

Conference Proceeding

Publication Date

6-27-2024

Publication Title

J Gen Intern Med

Keywords

alanine aminotransferase, aspartate aminotransferase, C reactive protein, corticosteroid, cyclosporine, methylprednisolone, prednisone, steroid, vancomycin, acute generalized exanthematous pustulosis, adult, adverse drug reaction, case report, clinical article, conference abstract, diagnosis, disease course, DRESS syndrome, drug exposure, drug therapy, drug withdrawal, emergency ward, eosinophil count, eosinophilia, erythema, excisional biopsy, face edema, female, follow up, human, human tissue, hypertransaminasemia, immunosuppressive treatment, intravenous drug administration, leukocyte count, lobular carcinoma in situ, middle aged, mortality rate, oral drug administration, prurigo, pustule, rash, scar tissue, severe cutaneous adverse reaction, side effect

Abstract

CASE: The patient underwent excisional biopsy of left breast lobular carcinoma in situ lesion and received intravenous vancomycin (1,250mg). Post-operative day (POD) 2, she developed a pruritic rash on her trunk, extremities, and face. Her surgeon prescribed oral methylprednisolone 4 mg POD 5. After no improvement, she presented to dermatology clinic on POD 8 where she was diagnosed with Acute Generalized Exanthematous Pustulosis (AGEP) secondary to vancomycin without biopsy as patient declined. Despite oral prednisone (40 mg daily), the rash worsened. She presented to emergency department POD 12 with elevated C-Reactive Protein (2.4 mg/dL), white blood cell count (22.3 K/uL), and absolute eosinophil count (3.1 K/uL). She had transaminitis with aspartate transaminase (AST) of 28 IU/L and alanine aminotransferase (ALT) of 74 IU/L. Facial edema, patchy erythema with pustules, and scattered purpuric patches were noted. Dermatology confirmed diagnosis of AGEP with overlapping Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome. Diagnosis was made with clinical/lab findings (no biopsy). She was admitted on POD 13 for worsening of above findings. IV methylprednisolone (1 mg/kg daily) was initiated. On POD 14, cyclosporine (175mg twice a day) was added in an effort to taper steroids due to patient's hesitancy with systemic corticosteroid use. On POD 19, her eosinophilia, rash and transaminitis all resolved or improved. Follow-up visits with allergy/immunology and dermatology showed no reactivation concerns. IMPACT/DISCUSSION: Compared to other medications, vancomycin is a rare but possible cause of severe cutaneous adverse reactions (SCARs). The presentation of AGEP is very rare with an estimated incidence of 1-5 cases per million per year. DRESS syndrome is also rare with an incidence of 1 in 1,000 to 1 in 10,000 per drug exposure. One study showed vancomycin to be responsible for a third of DRESS cases. Vancomycin-induced AGEP is highly uncommon; a 2015 report reveals only two previously reported cases in the literature. It is important to recognize the presentation of SCARs because of the potentially fatal outcomes and significant morbidity. Studies have shown DRESS syndrome to have a mortality rate of approximately 10%. In comparison, AGEP has a mortality rate of less than 5%. To the author's knowledge, this is the first reported case of AGEP and DRESS syndrome overlap due to vancomycin. In addition, the use of cyclosporine as an adjunct immunosuppressant contributes to this unique clinical course. Prompt, aggressive treatment with systemic steroids prevents morbid outcomes of systemic involvement and progression of these SCARs. CONCLUSION: - While rare, SCARs can present simultaneously. Distinguishing their presentations can prevent morbidity and mortality from commonly-used medications. - Organ involvement distinguishes DRESS syndrome from AGEP and warrants aggressive immunosuppressive treatment.

Volume

39

First Page

S410

Last Page

S411

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