Erythema Multiforme Is Not Always Erythema Multiforme

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Hana Nasim Ahmed M.D.
Meagan Mandabach Olivet
Carly Elston M.D.
Boni Elewski M.D.


medical dermatology, lupus, rowell syndrome, erythema multiforme, corticosteroids


Rowell syndrome (RS) describes the rare presentation of EM like lesions found in association with a diagnosis of lupus erythematosus (LE). Major diagnostic criteria includes LE, EM, and anti-nuclear antibodies. Minor criteria includes of chilblains, anti-Ro/anti-La antibodies, and rheumatoid factor. We present a 26-year-old white male seen in consultation for chronic erythema multiforme (EM) with a duration of 6 months unresponsive to treatment. Prior to our evaluation, he had been seen by another dermatologist and was diagnosed with biopsy-proven EM. His rash did not respond to previous treatment, including corticosteroids and antifungals. Lab results showed a positive ANA and positive anti-Ro antibody. The patient met diagnostic criteria for RS and was started on hydroxychloroquine 200 mg twice daily. Improvement was noticed two weeks after beginning treatment. Our case demonstrates that an atypical presentation of recurrent erythema multiforme, which does not respond to typical EM treatment, should raise a suspicion for RS and prompt screening for autoimmune markers and lupus erythematosus.





1. Gallo L, Megna M, Festa B, et al. Rowell Syndrome: A Diagnostic Challenge. J Clin Aesthet Dermatol. Apr 2020;13(4):40-42.

2. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. Jun 2013;27(3):391-404. doi:10.1016/j.berh.2013.07.008

3. Arevalo AB, Nassar R, Krishan S, Lakshmanan P, Salgado M, Chokshi P. Lupus Never Fails to Deceive US: A Case of Rowell's Syndrome. Case Rep Rheumatol. 2020;2020:8884230. doi:10.1155/2020/8884230

4. Anuj Sharma RS. Rowell Syndrome: A case report and review of literature`. Internet Journal of Rheumatology and Clinical Immunology. 1 September 2016 2016;4(1)

5. Devang Solanki ED, Nishita Drji. Case Report of a Rowell’s Syndrome. International Journal of Science and Research. 2014;3(1):7-8.

6. Callen JP, Klein J. Subacute cutaneous lupus erythematosus. Clinical, serologic, immunogenetic, and therapeutic considerations in seventy-two patients. Arthritis Rheum. Aug 1988;31(8):1007-13. doi:10.1002/art.1780310811

7. Muller CS, Hinterberger LR, Vogt T. Successful treatment of Rowell syndrome using oral cyclosporine A. Int J Dermatol. Aug 2011;50(8):1020-2. doi:10.1111/j.1365-4632.2010.04848.x

8. Lee A, Batra P, Furer V, Cheung W, Wang N, Franks A, Jr. Rowell syndrome (systemic lupus erythematosus + erythema multiforme). Dermatol Online J. Aug 15 2009;15(8):1.

9. Zeitouni NC, Funaro D, Cloutier RA, Gagne E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol. Feb 2000;142(2):343-6. doi:10.1046/j.1365-2133.2000.03306.x

10. Bolognia JL, Schaffer JV, Cerroni L, Callen JP. Chapter 41 Lupus Erythematosus. In: Dermatology. Edinburgh: Elsevier; 2018.

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