Rowell syndrome complicated with macrophage activation syndrome in a child

• Published in: Lupus Vol. 28; no. 14; pp. 1716 - 1721
• Main Authors: Aguirre-Martinez, I, Vélez-Tirado, N, García-Romero, M T, Rodríguez-Lozano, A L, Corcuera-Delgado, C T, Yamazaki-Nakashimada, M, Rivas-Larrauri, F
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.12.2019; Sage Publications Ltd
• Subjects: Autoantibodies; Cell activation; Child; Children; Diagnosis, Differential; Epidemiology; Erythema; Erythema multiforme; Erythema Multiforme - diagnosis; Erythema Multiforme - pathology; Humans; Lupus; Lupus Erythematosus, Systemic - diagnosis; Lupus Erythematosus, Systemic - pathology; Macrophage Activation Syndrome - etiology; Macrophages; Male; Pediatrics; Rare diseases; Rheumatoid factor; Skin - pathology; Systemic lupus erythematosus; hematologic changes; macrophage activation syndrome; Rowell syndrome; cutaneous lupus
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/0961203319886030

Rowell syndrome (RS) is a rare disease characterized by the association of systemic lupus erythematosus (SLE) or cutaneous lupus with lesions similar to erythema multiforme and the presence of autoantibodies including ANA, SSA, SSB, or rheumatoid factor. Due to the low incidence of this disease, the epidemiology of RS is not clear. So far there are 95 cases reported in the literature; of these, only seven cases are pediatric patients. Macrophage activation syndrome (MAS) is an increasingly recognized complication of SLE, although its true prevalence in childhood is still unknown. We describe a unique pediatric patient with RS who developed MAS.