Rowell syndrome in pregnancy: A diagnostic challenge

Abstract

Rowell syndrome is comprised of erythema multiforme-like lesions in association with lupus erythematosus along with serologies of speckled antinuclear antibodies, positive rheumatoid factor, positive anti-La/ anti-Ro and the clinical finding of chilblains. 1 Rowell syndrome is currently classified as a subtype of chronic cutaneous lupus erythematosus. 2 The majority of cases are seen in women aged 20-70 years. The ratio of affected women to men is 8:1. Indian patients have a median age of 23 compared to age 32 worldwide and are less likely to be discoid lupus. 3 We report a case of Rowell syndrome in pregnancy. A 29 year old primigravida at the period of gestation 15 weeks developed vasculitic lesions, erythema multiforme-like lesions in palms, soles, oral ulceration suggestive of LE lesions. She also had Alopecia universalis, Taenia corporaris and scabies masking the lesions of Rowell syndrome which was a diagnostic challenge. Laboratory investigations revealed ANA+1:640 speckled, Anti Ro+++, Anti La +++. The histology of the targetoid lesions was consistent with erythema multiforme. The patient was successfully managed on low-dose aspirin and hydroxychloroquine. Lesions showed significant improvement in one month. Early diagnosis, multidisciplinary team approach, and intensive monitoring during the antenatal period were key to the successful outcome of patient who had normal vaginal delivery of female child at term. This case reflects that we should suspect Rowell syndrome even in young patients without precipitating features and further cases must be reported to help increase knowledge of the pathophysiology of Rowell syndrome.