ANA-negative systemic lupus erythematosus with targetoid lesions: Rowell syndrome or just a rare presentation of lupus erythematosus
Keywords:
Lupus erythematosus, erythema multiforme, Rowell syndromeAbstract
Rowell syndrome is usually diagnosed by the presence of erythema multiforme (EM)-like targetoid lesions in the patients of cutaneous or systemic lupus erythematosus (CLE/SLE) and characterized by specific serological or immunological profile like speckled antinuclear antibody (ANA) pattern, antibody against saline extract of human tissues (anti-SjT) and positive serum rheumatoid factor (RF). The absence of universally accepted criteria and their poor specificity usually make the diagnosis very difficult. Moreover in the light of histopathological revelation of dermal mucin deposition along with interface changes and necrotic basal keratinocytes in the absence of serum positivity for ANA antibody and RF, true existence of this syndrome is still doubtful. Here we report a case of ANA-negative SLE having targetoid lesions on distal extremities and histopathological evidence of both the disease i.e LE and EM.References
Rowell NR, Beck SJ, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol. 1963;88:176-80.
Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: Does Rowell’s syndrome exist? J Am Acad Dermatol. 1999;40:773-7.
Zeitouni NC, Funaro D, Cloutier RA, Gagne E, Claveau J. Redefiing Rowell’s syndrome. Br J Dermatol. 2000;142:343-6.
Aydogan K, Karadogan SK, Balaban Adım S, Tunal. Lupus erythematosus associated with erythema multiforme: Report of two cases and review of the literature. J Eur Acad Dermatol Venereol. 2005;19:621-7.
Durmazlar SP, Oktay B, Eren C, Eskio lu F. So-called Rowell’s syndrome: Report of a case. J Turk Acad Dermatol. 2009;3:93201c
Aydin F, Senturk N, Yuksel EP, Yildiz L, Canturk T, Turanli AY. systemic lupus erythematosus with an erythema multiforme like lesions. Indian J Dermatol. 2007:52:56-8.
Pratap DSV, Reddy SK, Rani SC, Krishna VA, Indira D. ANA-negative systemic lupus erythematosus. Indian J Dermatol Venereol Leprol. 2004;70:243-4.
Khandpur S, Das S, Singh MK. Rowell's syndrome revisited: report of two cases from India. Int J Dermatol. 2005;44:545-9.
Dogra A, Minocha Y, Gupta M, Capalash P. Rowell's syndrome. Indian J Dermatol Venereol Leprol. 2000;66:324-5.
Bhat RY, Varma C, Bhatt S, Balachandran C. Rowell syndrome. Indian Dermatol Online J. 2014;5:33-5.
Sontheimer RD. Subacute cutaneous lupus erythematosus: 25-year evolution of a prototypic subset (subphenotype) of lupus erythematosus defied by characteristic cutaneous, pathological, immunological, and genetic findings. Autoimmune Rev. 2005;4:253-63.
James JA, Kaufman KM, Farris AD, Taylor-Albert E, Lehman TJ, Harley JB. An increased prevalence of Ebstein-Barr virus infection in young patients suggests a possible etiology for systemic lupus erythematosus. J Clin Invest. 1997;100:3019
Modi GM, Shen A, Mazloom A, Mawad J, Perez A, Sonabend ML et al. Lupus erythematosus masquerading as erythema multiforme: Does Rowell syndrome really exist? Dermatol Online J. 2009;15(2):5.
Lee A, Batra P, Furer V, Cheung W, Wang N, Franks A Jr. Rowell syndrome (systemic lupus erythematosus + erythema multiforme). Dermatol Online J. 2009;15(8):1.