(BMJ)—This patient presented with a 2-day hx of fever, headache, and new lesions on the hands, neck, and face. She had a background of systemic lupus erythematosus (SLE), for which she had been taking corticosteroids, azathioprine, and hydroxychloroquine for 2 years but was taking no other meds. Exam: erythematous, annular, target-like papuloplaques on the hands, face, and neck with lip ulcers. Serological tests for mycoplasma, HSV, cytomegalovirus, and EBV were negative. What’s the dx?
Rowell syndrome
erythema multiforme
toxic epidermal necrolysis
Stevens-Johnson syndrome
You are correct. The dx of Rowell syndrome requires the presence of 3 major criteria: erythema multiforme-like targetoid lesions, histological and serological evidence of SLE, and a speckled pattern of anti-nuclear antibodies, all present in this patient. At least 1 of the minor criteria must also be present for dx, including the presence of chilblains; anti-Ro(SS-A) (present in this patient) or anti-La(SS-B); and positive rheumatoid factor (present in this patient).
Rowell syndrome can mimic self-limiting erythema multiforme and lethal Stevens-Johnson syndrome or toxic epidermal necrolysis and should be suspected in patients with a diagnosis of SLE who present with targetoid lesions but without precipitating factors, such as infections, drugs, and malignancy. Its presence might indicate a flare-up of underlying SLE.
BMJ 2022;378:e070553