BG08 Treatment challenges in Rowell syndrome in older patients

Abstract

A 79-year-old woman presented with a 3-month history of a photodistributed rash, fatigue and nausea. She described a pruritic eruption predominantly involving the upper body. Clinical examination demonstrated discrete erythematous scaly patches on her face, chest and forearms, which coalesced into confluent eroded plaques on the upper arms. Additionally, multiple erythematous annular targetoid lesions were noted across the back and abdomen. Clinical examination revealed no mucosal lesions. No recent iatrogenic or infectious triggers were identified. Laboratory investigations revealed mild leucopenia and raised erythrocyte sedimentation rate. Anti-Ro, anti-La and antihistone antibodies were positive. Renal function was normal. Myositis panel and rheumatoid factor were negative. Skin biopsy from the back demonstrated vacuolar interface dermatitis with apoptotic keratinocytes, marked dermal oedema and perivascular lymphocytic infiltrate, consistent with erythema multiforme (EM). Skin biopsy from the forearm revealed compact orthokeratosis, perivascular lymphoid infiltrate, and increased dermal mucin, suggestive of cutaneous lupus erythematosus (LE). Given the presence of both LE and EM lesions alongside anti-Ro/anti-La positivity, a diagnosis of Rowell syndrome was determined. The patient commenced prednisolone 20 mg and hydroxychloroquine 400 mg daily alongside topical therapy, with resulting skin improvement. She had a prolonged hospital course, complicated by functional deconditioning and mobility decline, with an extensive deep vein thrombosis during admission. DEXA scan indicated osteopenia, and she requires ongoing low-dose systemic steroids. Upon discharge, she required assistance with mobility and activities of daily living, having previously lived independently with no supports. Rowell syndrome is a rare overlap of LE- and EM-like lesions with characteristic autoantibody positivity (anti-Ro, anti-La, and/or rheumatoid factor), and is rarely reported in older adults. Presentation at this age is therefore unusual and associated with distinct challenges including diagnostic delay, vulnerability to treatment toxicity and functional decline. This case underscores the substantial morbidity that rare autoimmune dermatoses may confer in older patients.