Lupus Erythematosus and Erythema Multiforme-like Lesions

Abstract

erythema multiforme-like (EM) lesions, positive speckled ANA, RF and Anti-SJT antibody, later known as anti Ro/La antibodies. In 2000, Zeitouni et al extended the definition by including all types of systemic lupus erythematosus (SLE) and proposed major and minor criteria, while in 2012 Torchia et al proposed another set of criteria defining Rowell syndrome as a distinct subtype of chronic cutaneous lupus. To date, Rowell syndrome as a distinct entity remains in question. Objectives: To establish whether patients with clinically suggestive Rowell syndrome, meet the proposed criteria and form a distinct SLE subgroup. Methods: A retrospective study which included SLE patients who associated EM-like lesions was carried out in the Rheumatology Department Cluj-Napoca, between 2008 and 2019. Clinical, immunological and histopathological parameters were recorded. Results: Among 200 patients who fulfilled the 2012 SLICC criteria, 12 patients with target lesions, resembling EM, were identified. Four patients were excluded: 3 were not fully investigated and 1 was My. pneumoniae positive, thus 8 patients were studied. The majority of patients (87.5%) developed the cutaneous lesions after diagnosis. In all cases, the erythematous maculopapular rash with targetoid aspect was present, with poorly defined borders and a dusky center and a diameter between 2 to 6 cm. The lesions extended to the trunk and limbs, sparring the acral and mucosal areas. Five patients associated pruritus. The lesions could not be linked to any viral or bacterial infection in any of the cases. With regard to drug allergies, a link with AZA and HCQ was suspected in three patientswhich was infirmed. The majority of patients (87.5%) had negative anti-ds DNA, 87.5% presented speckled pattern ANA, one patient exhibited ANA rods and rings pattern, with a negative serology for B and C hepatitis and no antiviral therapy. Of note, 25% had positive rheumatoid factor, 87.5% had positive anti-Ro antibody and 37.5% of patients exhibited chilblains. The first three classifications were tested on all patients: 25% met Rowell's criteria, 12.5% met Lee's criteria and 87.5% met Zeitouni's criteria, with only 1 patient meeting all 3, and 1 patient meeting none of them. In the cases in which the SLE etiology of the lesion was in question from a clinical point of view, a biopsy was performed. In one case the histopathological examination described lymphocytic infiltrates and few eosinophils in the dermis, suggestive of drug allergy. Interestingly, this patient met all 3 classification criteria. Another report described thin epidermis, parakeratosis, dyskeratosis and spongiosis with dermis and perivascular lymphocytic infiltrate-findings highly suggestive for EM; this report belonged to the patient who met neither of the 3 classifications, but who associated a rods and rings ANA pattern. The third histopathological report was inconclusive. Conclusion:Our study shows that patients with suspected clinical, immunological or histological Rowell syndrome do not meet all studied c...

Drug-Induced, Ro/SSA-Positive Cutaneous Lupus Erythematosus

“…2003;139:45-49 S UBSETS OF cutaneous lupus erythematosus (CLE ) associated with the presence of anti-Ro/SSA antibodies include the following [1][2][3][4][5][6][7][8][9] : (1) drug-induced lupus erythematosus (LE); (2) neonatal LE; (3) LE with inherited C2 and C4 deformities; (4) LE in the elderly; (5) Sjö gren syndrome; (6) LE and benign hypergammaglobulinemic purpura of Waldenstrom syndrome (lymphocytic vasculitis); (7) LE and photosensitivity; (8) subacute CLE (SCLE); and (9) LE with erythema multiformelike lesions (Rowell syndrome). The most recent and least characterized subset of LE has been the phenomenon of drug-induced Ro/SSApositive CLE.…”

Rowell’s syndrome: a Case Report and Review of Literature

“…In 1963, Rowell et al reported a unique syndrome characterized by SLE, EM-like lesions, a positive test for RF, speckled ANA and a saline extract of human tissue (anti-SJT) which is now known as similar to Ro (SSA) [1]. Since the first report of Rowell's syndrome very few cases have been reported in the literature in which the presence of EM-like lesions are associated with LE.…”

“…In 1963, Rowell defined this association as a distinct entity upon discovering different clinical and immunologic findings in four patient during his study including 120 discoid lupus erythematosus (DLE) patients. The original criteria of Rowell's syndrome comprised of LE, EM like lesions and immunological abnormalities such as speckled pattern of ANA, RF and saline extract of human tissue (anti-SJT) positivity which is now regarded as similar to anti-Ro [1]. Although this syndrome was originally described in DLE patients, some of these patients developed SLE years after the onset of DLE [3].…”

“…In the year 2000, Zeitouni et al came up with major and minor criteria [4]. Rowell's syndrome has been reported with all subtypes of LE (systemic, acute, subacute or discoid) [1,4,5]. Conversely, all forms of EM (EM minor, EM major) can be associated with Rowell's syndrome [5,6].…”

“…Rowell's syndrome is a rare presentation of lupus erythematosus (LE) with erythema multiforme (EM) like lesions in association with antinuclear antibody (ANA), anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity [1]. Cases of Rowell's syndrome have been rarely encountered in literature, since its first description which was way back in 1963.…”