Case Presentation: 38-year-old female with past medical history of SLE and chronic cutaneous lupus was transferred from a community hospital for evaluation of progressively worsening rash. Patient was recently started on a Z-Pak for URI and Diflucan for yeast infection. She started experiencing itching around her neck and the following day noticed a rash around her neck, arms and upper chest which progressed leading for her to seek evaluation at urgent care. Next day, she was seen in dermatology clinic and prescribed triamcinolone ointment along with triple antibiotic cream. Her rash continued to worsen and started involving intertriginous areas of her groin which led her to seek ED evaluation. She was treated with supportive therapy along with Foley placement for comfort. Rash was erythematous to violaceous plaques and some flaccid bullae on face, chest, and arms, Upper eyelids with some erosions with yellow crust. Eroded plaques bilateral inguinal fold. No involvement of soles or palms. She lacked systemic symptoms including fever chills eye irritation, pain, joint pain, vomiting, diarrhea or oral ulcers. Her skin lesions were different than her previous lupus flares. She denied over-the-counter medications, supplements or any new prescriptions.Differential diagnosis at that point were drug reaction, subacute cutaneous lupus versus bullous lupus, Erythema multiforme.Serologies including HSV,HIV negative. Respiratory viral panel was negative. Previous Rheumatological workup: dsDNA ab, Rheumatoid Factor, CCP antibody, APS panel were all within normal limits, SSA/SSB-ab, anti-Smith, anti-RNP all elevated. Dermatology was consulted and performed a punch biopsy. Pathology results shows strips of necrotic epidermis with a superficial and deep lymphoplasmacytic perivascular and periadnexal inflammation. Given the clinical manifestations, lab testing, histopathological findings the diagnosis of Rowell syndrome was made. Patient was managed with topical and systemic steroids. Patient showed improvement. Patient was discharged home on steroid. It is thought to be triggered by her recent URI.
Discussion: It is highly debated in literature whether it is an overlap syndrome, a real association or coincident of discoid Lupus erythematous and Erythema multiforme. Given the controversy, the diagnostic criteria has been modified. Rowell syndrome is a rather rare disease consisting of erythema multiforme like lesions in patients with lupus. The major diagnostic criteria for Rowell syndrome are the presence of lupus erythematosus (acute, subacute or systemic), erythema multiforme-like lesions and positive testing for antinuclear antibodies. The minor diagnostic criteria for Rowell syndrome are chilblains, the presence of SS-A antibodies and positive testing for rheumatoid factor. All the major criteria and at least 1 of the minor criteria required to confirm the diagnosis of Rowell syndrome.Most of the cases have been reported in middle-aged woman. Speckled ANA pattern is the most consistent feature of RLS occurring in about 88% of cases, whereas RF is least preserve future president only 41%.Both SLE and Rowell syndrome respond to similar therapeutic regimen. Most of the cases has shown good response to steroids, azathioprine or hydroxychloroquine. Our patient responded well to steroids.
Conclusions: In conclusion, Rowell syndrome is a rare syndrome. It should be suspected in all patients with SLE with EM-like lesions with no evidence of any precipitating factor for EM.