Drug-induced Rowell syndrome, a rare and difficult to manage disease: A case report

Abstract

Rowell syndrome is defined as the association between lupus erythematosus, erythema multiforme-like lesions and characteristic immunological changes including positive tests for rheumatoid factor, speckled antinuclear antibody, positive anti-Ro or anti-La antibodies. The present report presents the case of a 45-year-old female patient who was previously diagnosed in January 2010 with terbinafine-induced subacute cutaneous lupus erythematosus and was admitted for a skin eruption consisting of erythematous-papular erythema multiforme-like lesions, primarily on the trunk and limbs. The associated symptoms consisted of fatigability, myalgia and gonalgia. In October 2015, the illness reoccurred ~1 week after the initiation of Helicobacter pylori eradication treatment. Anti-Ro antibodies, rheumatoid factor and antinuclear antibody tests were positive. Given the patient's medical history, clinical manifestations, and laboratory, histopathological and immunofluorescence microscopy findings, a diagnosis of Rowell syndrome was made. Systemic corticosteroids (methylprednisolone; 0.5 mg/kg/day) and immunomodulatory therapy (azathioprine; 50 mg/day) were administered with the associated medication (omeprazole, 20 mg/day; KCl, 1 g/day) and topical dermocorticoids (fluticasone propionate 0.05% cream; 1 application/day), with a favorable outcome. 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 anti-Ro antibodies and positive testing for rheumatoid factor. A diagnosis of Rowell syndrome is made if the patient exhibits all major criteria and at least one minor criterion. The present case met all diagnostic criteria, excluding the presence of chilblains. Notably, in this case there was a co-occurrence of subacute lupus erythematosus and Rowell syndrome lesions, which was drug-induced.

Keywords: Rowell syndrome; erythema multiforme-like; lupus erythematosus.

Figure 1.

Representative image of the skin lesions observed, en cocarde covered by scales that were only adherent in the center of the lesions and exhibited a pale center on the posterior trunk.

Figure 2.

Miscrocopy observation of lesion biopsies. (A) Hematoxylin and eosin staining. Magnification, ×100. (B) Positive complement component 3 staining via direct immunofluorescence. Magnification, ×100.

Figure 3.

Favorable outcome following treatment and subsequent relapse. (A) Skin lesions on the posterior trunk improved. (B) Relapse of the lesions en cocarde on the face.