Lupus Never Fails to Deceive US: A Case of Rowell’s Syndrome

Abstract

Background. Rowell’s syndrome is comprised of the presentation of erythema multiforme- (EM-) like lesions in association with lupus erythematosus (LE), along with serologies of speckled antinuclear antibodies (ANAs), positive rheumatoid factor (RF), positive anti-La/anti-Ro, and the clinical finding of chilblains. As per the redefined criteria by Zeitouni et al., three major criteria in addition to at least 1 minor criterion are necessary for diagnosis. Case Presentation. A 20-year-old male presented with a one-week history of worsening nonpruritic erythematous maculopapular skin rash (resembling EM) which appeared on the face and subsequently spread to the trunk, arms, legs, palms, and soles. There was no mucosal involvement. At the onset of rash, the patient reported headaches, associated with photosensitivity and intermittent fevers. Workup for viral meningitis yielded negative results. Laboratory investigation revealed mild anemia, elevated inflammatory markers, a positive ANA with speckled pattern, a positive anti-Ro/SSA, anti-La/SSB antibodies, and a positive rheumatoid factor (RF). Lupus anticoagulant antibody was positive along with a low-positive anticardiolipin IgM antibody and a negative beta-2 glycoprotein antibody. Anti-dsDNA, anti-Smith, anti-Jo-1, anti-centromere, and anti-Scl-70 antibodies were negative. Hepatitis serologies, herpes simplex virus 1 and 2, mycoplasma, Epstein–Barr virus, HIV, and parvovirus B19 were negative. Left arm skin biopsy demonstrated vacuolar interface dermatitis and positive colloidal iron stain suggestive of dermal mucin deposition, favoring the diagnosis of cutaneous collagen vascular disease. Cutaneous lesions improved with administration of oral prednisolone. Conclusion. Rowell’s syndrome should be considered in patients who present with cutaneous LE and lesions resembling EM. Further serological markers should be pursued in the absence of obvious EM-precipitating factors.

Systemic lupus erythematosus associated with erythema multiforme: A rare case report of Rowell's syndrome

“…Also, a diagnosis of RS is based on the presence of major and minor criteria, as seen in Table 2. 1,5,6,11,14 All three major and at least one minor criteria are required to confirm RS. 6 In our patient, as all the major criteria, along with one minor criterion, that is, positive RF, were present, we consider our case to be a classic RS.…”

“…Also, a diagnosis of RS is based on the presence of major and minor criteria, as seen in Table 2 . 1 , 5 , 6 , 11 , 14 All three major and at least one minor criteria are required to confirm RS. 6 …”

Erythema Multiforme Is Not Always Erythema Multiforme

“…Though LE is a major diagnostic criterion of RS, another case report by Arevalo et al documented a case of a 20 year-old male with no prior LE diagnosis. 3 Clinicians evaluating an atypical presentation of EM should keep RS in mind regardless of a previous diagnosis of LE, refer their patients for the screening of LE, and order appropriate serology.…”

“…7 Management for RS includes the use of steroids and immunosuppressive drugs, including azathioprine and cyclosporine. 3,4,7 Antimalarials, such as hydroxychloroquine and chloroquine, have also been employed, as well as dapsone. 1,4,8,9 Our patient has responded well to hydroxychloroquine.…”

Drug Induced Rowell’s Syndrome: A Rare Presentation

“…Erythema multiforme-like lesions as the initial presentation in a patient with systemic lupus erythematosus (SLE / DLE) is a rare finding. 1,2 Rowell's syndrome (RS) was originally proposed in 1963 by Rowell et al 3 This criterion was revised by Zeitouni et al 4 in the year 2000. Major criteria included the coexistence of lupus erythematosus or subacute cutaneous lupus erythematosus with EM-like lesions with or without mucosal involvement and speckled pattern of ANA.…”