Fever, rash, and leukopenia in a 32-year-old man • Dx?

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Case Reports

Fever, rash, and leukopenia in a 32-year-old man • Dx?

J Fam Pract. 2017 October;66(10):E7-E10

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References

1. Cacoub P, Musette P, Descamps V, et al. The DRESS syndrome: a literature review. Am J Med. 2011;124:588-597.

2. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome: Part I. Clinical perspectives. J Am Acad Dermatol. 2013;68:693.e1-e14.

3. Bourgeois GP, Cafardi JA, Groysman V, et al. Fulminant myocarditis as a late sequelae of DRESS-2 cases. J Am Acad Dermatol. 2011;65:889-890.

4. Cho YT, Yang CW, Chu CY. Drug reaction with eosinophilia and systemic symptoms (DRESS): an interplay among drugs, viruses, and immune system. Int J Mol Sci. 2017;18:1-21.

5. Alfirevic A, Pirmohamed M. Drug-induced hypersensitivity and the HLA complex. Pharmaceuticals (Basel). 2011;4:69-90.

6. American College of Rheumatology. 1997 Update of the 1982 American College of Rheumatology Revised Criteria for Classification of Systemic Lupus Erythematosus. Available at: https://www.rheumatology.org/Portals/0/Files/1982%20SLE%20Classification_Excerpt.pdf. Accessed August 30, 2017.

7. Descamps V, Ben Saïd B, Sassolas B, et al. Management of drug reaction with eosinophilia and systemic symptoms (DRESS). Ann Dermatol Venereol. 2010;137:703-708.

8. Peyrière H, Dereure O, Breton H, et al. Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist? Br J Dermatol. 2006;155:422-428.

9. Pichler WJ, Tilch J. The lymphocyte transformation test in the diagnosis of drug hypersensitivity. Allergy. 2004;59:809-820.

10. Husain Z, Reddy BY, Schwartz RA. DRESS syndrome part II: management and therapeutics. J Am Acad Dermatol. 2013;68:709.e1-e9.

11. Funck-Brentano E, Duong TA, Bouvresses S, et al. Therapeutic management of DRESS: a retrospective study of 38 cases. J Am Acad Dermatol. 2015;72:246-252.

DISCUSSION

DRESS is typically characterized by fever, rash, eosinophilia, atypical lymphocytes, lymphadenopathy, and organ involvement (primarily liver, but multiple organ systems can be affected).¹ Patients with severe symptoms have renal involvement, anemia, respiratory and cardiac symptoms (chest pain, tachycardia, and myocarditis), and transaminase levels up to 5 times greater than normal.^1-3 Anticonvulsants and antibiotics are the most common offending classes among the medications that are associated with DRESS (TABLE 1).^2,4

The reported incidence of DRESS is between one in 1000 and one in 10,000 drug exposures.¹ Due to the broad presentation and a lack of established diagnostic criteria associated with DRESS, this number may be even higher. DRESS has a 10% mortality rate,¹ and hepatic necrosis is the most common cause of death.²

Certain people may be more prone to DRESS. People with certain gene mutations that code for drug detoxification enzymes have shown a greater incidence of DRESS.⁵ Viral reactivation, commonly of HHV-6, has also been shown to have an effect on the pathogenesis of DRESS. Additionally, genetic predisposition involving specific human leukocyte antigens (HLAs) makes certain people more prone to the development of DRESS (TABLE 2).^2,5

Case reports have demonstrated a link between certain autoimmune syndromes and DRESS, specifically Grave’s disease and type 1 diabetes mellitus.²

Patch testing and lymphocyte transformation tests can aid in the diagnosis of DRESS.

A unique finding of this case was the presence of elevated ANA and anti-Sm antibody titers at initial presentation, with spontaneous negative seroconversion 2 months later. Because of these 2 findings, as well as the patient’s leukopenia and rash, he briefly met 4 of the 11 criteria set forth by the American College of Rheumatology for a diagnosis of systemic lupus erythematosus (SLE).⁶ It is unclear whether the transiently elevated anti-Sm antibody titers were an acute phase reactant due to DRESS, a viral illness, or an evolving autoimmune process.

The false-positive rate for anti-Sm antibodies in association with DRESS has not been previously reported.

Fever, rash, and leukopenia in a 32-year-old man • Dx?

References

DISCUSSION

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