<p>A Case of Rhupus with Rowell Syndrome</p>

Abstract

We report a case of 30-year-old female who presented initially with hair loss, photosensitive malar rash, morning stiffness and synovitis. She was diagnosed with Rhupus syndrome based on clinical and laboratory findings. Few months after starting hydroxychloroquine, esomeprazole and azathioprine, and failing methotrexate (because of erosive pillinduced esophagitis), she presented with generalized maculopapular dusky reddish rash in her body, back and extremities. Her anti-double stranded-DNA, anti-nuclear antibody, anti-Ro/SSA and anti-La/SSB were positive. Anti-cyclic citrullinated peptide antibody was moderately positive. She had low complements: C3 and C4. Herpes simplex IgM and mycoplasma tested negative. Skin biopsy from right arm showed evidence of erythema multiform. She met the criteria for the diagnosis of Rowell syndrome. We managed her with hydroxychloroquine, prednisolone, mycophenolate mofetil and topical agents and discontinued esomeprazole. We also review the management of Rowell syndrome in the literature.

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

“…Major criteria include the presence of systemic or cutaneous lupus erythematosus (CLE), erythema multiform‐like lesions, and antinuclear antibodies (ANA) with speckled pattern. Minor criteria include the presence of chilblains, anti‐Ro, or anti‐La antibodies or rheumatoid factor (RF) 4 . The appearance of the speckled pattern of ANA is the most reliable and consistent factor in the diagnosis of RS 1,6 .…”

“…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.…”

“…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 , 2 , 3 , 4 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).…”

“…Minor criteria include the presence of chilblains, anti‐Ro, or anti‐La antibodies or rheumatoid factor (RF). 4 The appearance of the speckled pattern of ANA is the most reliable and consistent factor in the diagnosis of RS. 1 , 6 While the precise etiopathogenesis of RS remains unclear, it is believed to be triggered by factors such as drugs, infections, ultraviolet exposure, cigarette smoking, and psychological stress.…”

Drug Induced Rowell’s Syndrome: A Rare Presentation

“…4 Proton pump inhibitors were described in the literature for drug-induced RS. [5][6][7] We describe a rare overlap syndrome of SLE and EM-like skin lesions.…”