Systemic Lupus Erythematosus Associated with Erythema Multiforme-Like Lesions

Erythema multiforme (EM) and systemic lupus erythematosus (SLE) are common diseases. Their coexistence is known as Rowell syndrome (RS), first described in 1963. Only few cases of RS have been described and some of them questioned its existence. We present two cases of SLE in the setting of a newly developed EM-like eruption, which shares many similarities with the so-called Rowell syndrome. 1. Introduction Systemic lupus erythematosus (SLE) has been rarely associated with erythema multiforme- (EM-) like eruption. In 1963, Rowell et al. described a distinctive subset of patients diagnosed with discoid lupus erythematosus (DLE) associated with EM and a characteristic pattern of immunological abnormalities (Table 1) [1]. We report two cases of SLE in the setting of a newly developed EM-like eruption, which shares many similarities with the so-called Rowell syndrome (RS). Table 1 Case 1. A 29-year-old African-American woman, with history of stroke and seizure disorder, presented with a 2-week history of diffuse rash without systemic symptoms and no apparent precipitating event. She had been taking phenytoin 100？mg three times daily for 2 years. Physical examination revealed scarring hypopigmented alopecia with follicular plugging and adherent scale extending from the ear lobes to the occipital scalp, clinically consistent with discoid lupus erythematosus (DLE) (Figure 1). There were also scattered erythematous to violaceous papules and plaques, mostly annular, some with scales, which coalesced into large polycyclic lesions, present on the chest, abdomen, arms, and legs with an overall appearance of subacute cutaneous lupus erythematosus (SCLE) (Figure 2). There were no oral, genital, or ocular lesions. The clinical impression indicated the coexistence of DLE and SCLE. However, a subsequent 4？mm punch biopsy of the SCLE-like lesions revealed histological hallmarks of erythema multiforme (EM), including perivascular lymphocytic infiltrate and widespread keratinocyte necrosis. Further workup demonstrated a 1？:？640 antinuclear antibody (ANA) with speckled pattern, anti-SSA > 8 (normal < 1), anti-SSB > 8 (normal < 1), C3 27 (normal 86–184？mg/dL), and C4 6 (normal 20–59？mg/dL). Rheumatoid factor was negative. A course of methylprednisolone at a dose of 120？mg IV daily was initiated. Hydroxychloroquine 400？mg daily was added. The lesions started to resolve 3 days later and the dose of methylprednisolone was gradually tapered within a period of 5 weeks. Figure 1 Figure 2 Case 2. A 46-year-old Hispanic woman presented with 1-month history of widespread skin rash