Abstract

Rowell Syndrome (RS) is a rare disease first reported in 1963 as a clinical and histopathological overlap of Lupus Erythematosus (LE) and Erythema Multiforme (EM) with characteristic serological findings. Zeitouni et.al. suggested that 3 major criteria (LE, EM-like lesions, and speckled pattern of antinuclear antibodies) and 1 of 3 minor criteria (chilblains, anti-Ro/La antibodies, and rheumatoid factor) are needed for a diagnosis. Subsequent publications expressed doubt about the uniqueness of these findings and considered RS as a variant of LE. Our patient is a 77-year-old female with systemic LE and Sjogren’s syndrome who presented with a pruritic rash involving her forearms and upper back. It is composed of scaly round pink plaques with central erosions and is occasionally polycyclic. Biopsy showed an orthokeratotic corneum overlying areas of epidermal necrosis. Adjacent viable epidermis demonstrated basal vacuolar changes and dyskeratotic keratinocytes at all levels. Other areas had compact hyperkeratosis with follicular plugging and a mild superficial and mid dermal lymphocytic infiltrate. Subsequent testing revealed a positive speckled ANA, RF, and SSA/SSB meeting all criteria for RS. The patient was treated with mycophenolate mofetil and topical steroids with improvement of her rash. Whether RS is a distinct entity or a variant of LE, it remains important to recognize this rare pattern and avoid misdiagnosing it as EM. The latter, oftentimes, is self-limiting and requires supportive care, whereas most successful RS treatments combined corticosteroids with immunosuppressive therapy (methotrexate, dapsone, hydroxychloroquine, and azathioprine) which is aimed at the underlying LE.