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

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.

Rowell’s syndrome: a rare but distinct entity in rheumatology

Rowell’s syndrome is a rare disorder characterised by an association of lupus erythematosus with erythema multiforme (EM)-like skin lesions. EM as the initial clinical presentation of systemic lupus erythematosus is also atypical and even rarer. We report the case of an 18-year-old girl admitted to our hospital with fever and polyarthralgia along with multiple discrete ill-defined target lesions with crust formation over forehead, cheek, external ears, scalp, upper chest and back (predominantly over sun-exposed areas) with ulceration over hard palate. Investigations revealed pancytopaenia, a positive rheumatoid factor, positive antinuclear antibody with a speckled pattern, anti-Smith antibody and strongly positive anti-Ro. Patient was diagnosed with Rowell’s syndrome as per clinical and laboratory features. Majority of skin lesions including oral ulcerations subsided gradually after treatment with steroids and hydroxychloroquine.

Cutaneous manifestations and systemic correlation in patients with lupus erythematosus and its subsets: a study of 40 cases

<p class="abstract"><strong>Background:</strong> The objective was to study various dermatological manifestations and clinical and laboratory features in patients of lupus erythematosus (LE) and its subsets.</p><p class="abstract"><strong>Methods:</strong> This is a cross sectional observational study done in a tertiary care hospital in a rural setup in Piparia, Ta. Waghodiya, Dist. Vadodara, Gujarat. All the patients with clinical features of LE and its subsets were included in the study over a period of 16 months and were subjected to detailed history taking, complete cutaneous and general examination and laboratory investigations.<strong></strong></p><p class="abstract"><strong>Results:</strong> Out of the 40 cases, 22 cases were systemic lupus erythematosus (SLE), 13 cases were discoid lupus erythematosus (DLE), four cases were of Rowell’s syndrome and one case was of mixed connective tissue disorder (MCTD). 97.5% of cases had cutaneous involvement, i.e. photosensitivity (77.5%), oral ulcers, hair loss and malar rash. Systemic symptoms and abnormal laboratory parameters were present in the cases of SLE, Rowell’s syndrome and MCTD, with hematological involvement being the most common in both SLE (77%) and Rowell’s syndrome (100%) and positive anti-nuclear antibody (ANA) titer being the most common abnormal laboratory finding in both SLE (95.4%) and Rowell’s syndrome (100%). The most commonly found antibody was anti-dsDNA (64.3%) in SLE and anti SS-A (100%) in Rowell’s syndrome.</p><p class="abstract"><strong>Conclusions:</strong> Cutaneous features though occasionally subtle, are pointers to a diagnosis of SLE. Hair loss, malar rash and photosensitivity alone or in association with altered hematologic/ANA profile are the key markers of the disease activity.</p><p> </p>