(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?
toxic epidermal necrolysis
Rowell syndrome
Stevens-Johnson syndrome
erythema multiforme
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