Erythema Multiforme Is Not Always Erythema Multiforme

Hana Ahmed,Carly Elston,Boni Elewski,Meagan Olivet

doi:10.25251/skin.6.1.10

Hana Ahmed, Carly Elston + Show 2 more

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https://doi.org/10.25251/skin.6.1.10

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Abstract

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.  

Highlights

Rowell syndrome (RS) describes the rare presentation of erythema multiforme (EM) like lesions found in association with a diagnosis of lupus erythematosus (LE)
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
RS describes the rare presentation of EM like lesions found in association with a diagnosis of lupus erythematosus (LE)

Summary

INTRODUCTION

Rowell syndrome (RS) describes the rare presentation of EM like lesions found in association with a diagnosis of lupus erythematosus (LE). Preliminary lab work was ordered, including a complete blood count (CBC), a comprehensive metabolic panel (CMP), and an antinuclear antibody (ANA). The ANA was positive, and he was pending a consult with rheumatology. Given the positive ANA and Anti-Ro, a diagnosis favoring Rowell Syndrome (RS) was made and the patient was started on hydroxychloroquine 200 mg twice daily. After two weeks after initiation of this treatment, the patient began to note improvement of his rash. Additional lab work was obtained and included CBC, CMP, glucose-6-phosphate dehydrogenase, T-spot test, acute hepatitis panel, flow cytometry, a dermatomyositis panel, ANA, anti-Ro, and Anti-La. The labs were within normal limits except for a repeated positive ANA (1:320 with a speckled pattern) and positive anti-Ro antibody. The DIF was negative and H&E staining indicated an interface dermatitis with prominent dyskeratotic cells

DISCUSSION

CONCLUSION