Targetoid‐like lesions and chilblain‐like erythema manifested on hands and feet: A case of Rowell syndrome from China

Abstract

Background: Rowell syndrome (RS) is an uncommon condition characterized by erythema multiforme (EM)‐like lesions and lupus erythematosus. It is more common in females, and EM may be the first manifestation of the disease with positive autoantibodies, such as antinuclear antibody (ANA), SSA, SSB and rheumatoid factor. The pathogenesis of RS is unknown and is likely caused by drug induction, ultraviolet exposure and infection. Method: We describe a case of RS from China which presented as characteristic targetoid‐like lesions and chilblain‐like erythema on hands and feet. This is a case of RS in a female patient from the inpatient department of dermatology. Results: A 41‐year‐old female with systemic lupus erythematosus exhibited chilblain‐like erythema and characteristic EM lesions on her extremities. She tested positive for serum ANA (1:320) and anti‐double‐stranded DNA, as well as other autoantibodies. Systemic glucocorticoids and hydroxychloroquine worked effectively for her. Conclusion: The present case met diagnostic criteria of RS. Notably, there was a co‑occurrence of facial butterfly erythema, chilblain‐like erythema and EM lesions distributed on the limbs in this case.

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

“…1,11,13 Studies have shown that Belimumab, a human monoclonal antibody directed against the B lymphocyte stimulator, is often considered effective in recalcitrant cases of SLE, although data regarding its use in cutaneous SLE is limited. 1 The response to treatment can be assessed using the levels of certain au-dsDNA, anti-Nucl, anti-His, and anti-C1q), among which anti-dsDNA performs the best when assessing disease activity, as suggested by the researchers Shang et al 3 Our patient was subsequently managed with hydroxychloroquine (200 mg once daily), prednisolone (1 mg/kg/day), steroid ointment, a proton pump inhibitor, and sunscreen cream after the diagnosis. Following the treatment, the skin lesions gradually resolve, as shown in Figures 1-3.…”

“…The therapeutic regimen used for RS and the prognosis are similar to those of SLE or DLE that occurs alone. However, the diagnosis becomes challenging when DLE coexists with SLE 3 . The majority of the reported cases showed a satisfactory response to corticosteroids with azathioprine, antimalarial drugs such as chloroquine or hydroxychloroquine, dapsone, or cyclosporine 1,11,13 .…”

“…EM is distinguished by evident target lesions on the skin, accompanied by erosion, blisters, or bullae of mucosal areas (such as the mouth, genitals, and eyes). 3,5 To reach a diagnosis, meeting all major criteria along with one minor criterion is necessary. Major criteria include the presence of systemic or cutaneous lupus erythematosus (CLE), erythema multiform-like lesions, and antinuclear antibodies (ANA) with speckled pattern.…”

“…Rowell's syndrome (RS) is an uncommon entity in which patients with systemic lupus erythematosus (SLE) rarely develop characteristic lesions similar to those of erythema multiforme (EM)‐like skin lesions, in the presence of specific serological abnormalities 1–4 . Firstly, the association between lupus and EM was described by Scholtz in 1922.…”

“…Firstly, the association between lupus and EM was described by Scholtz in 1922. In 1963, Professor Neville Rowell and his colleagues reported four female patients with discoid lupus erythematosus (DLE) and EM‐like skin lesions (among 120 patients with DLE) 2,3 . SLE is a chronic autoimmune‐mediated inflammatory disorder with multisystem and multiorgan manifestations, while EM is an acute, immune‐mediated condition linked to infection, medications, and autoimmune disorders without special autoantibodies, as seen in autoimmune disorders.…”